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A  TREATISE 


ON  THE 


NERVOUS  DISEASES  OF 

CHILDKEN 

FOR 

PHYSICIANS  AND   STUDENTS 


BY 

B.    SACHS,    M.D. 

ALIENIST  AND  NEUROLOGIST  TO  BELLEVUE  HOSPITAL;   NEUROLOGIST  TO  THE  MT.  SINAI  HOSPITAL; 

CONSULTING  PHYSICIAN    TO  MANHATTAN  STATE  HOSPITAL  EAST,   AND  WEST;    FORMERLY 

PROFESSOR  OF  MENTAL  AND  NERVOUS  DISEASES  IN  THE  NEW  YORK  POLYCLINIC; 

EX-PRESIDENT  OF  THE  AMERICAN  NEUROLOGICAL  ASSOCIATION 


SECOND  EDITION,  REVISED 


NEW  YORK 

WILLIAM    WOOD   AND    COMPANY 

MDCCCCV 


Copyright,  1905,  by 
WILLIAM  WOOD  AND  COMPANY 


PREFACE    TO    SECOND    EDITION. 


The  author  has  reason  to  be  pleased  with  the  praise 
bestowed  upon  the  first  edition  of  his  book  in  this  country 
and  in  Europe.  Translations  into  German  and  Italian  have 
helped  to  extend  its  teachings,  and  a  French  edition  is  to 
appear  shortly.  Recent  writers  of  text-books  on  Medicine 
and  on  Pediatrics  have  made  liberal  use  of  it.  It  is  evident 
that  the  book  has  filled  a  distinct  gap  in  medical  literature. 

In  preparing  the  second  edition  the  writer  has  endeav- 
ored to  reduce  the  size  of  the  book  without  lessening  its 
value  as  a  special  treatise  on  the  nervous  diseases  of  chil- 
dren. He  has  omitted  the  chapters  on  Anatomy  and 
Physiology,  the  subject  matter  of  which  can  readily  be 
found  in  the  text-books  of  Dana,  Gowers,  Starr  and  Oppen- 
heim.  All  detailed  histories  of  cases  and  all  bibliographies 
have  been  omitted.  The  names  of  authors  have,  however, 
been  inserted  in  the  text  to  give  credit  for  work  done, — 
a  bit  of  justice  which  a  writer  should  be  happy  and  ready 
to  do  to  others. 

Everv  chapter  has  been  carefully  revised  and  much  new 
matter  has  been  introduced.  A  comparison  of  the  two 
editions  of  this  book  will  show  that  important  advances 
have  been  made  in  Neurology  and  the  allied  sciences  with- 
in recent  years. 

The  author  wishes  to  express  his  especial  thanks  to  his 
publishers  for  their  interest  in  this  new  edition,  and  to 
Dr.  Alfred  Wiener,  of  this  City,  for  his  assistance  in  pre- 
paring the  index. 

21  East  Sixty-fifth  Street. 
June,  ipoj. 


CONTENTS. 


CHAPTER    I. 

PAGE 

Introduction  —  Methods  of  Examination, i 

Examination  schemes,  3;  cranial  measurements,  5;  Preyer's  observations, 
7;  visual  tests,  8;  action  of  muscles,  11-34;  gait,  35;  sensory  distribu- 
tion, 36;  reflexes,  40;  electrical  examinations,  42;  lumbar  puncture,  46. 


PART    I. 

GENERAL    NERVOUS    DISEASES. 


CHAPTER   II. 

Convulsions  —  Eclampsia  Infantum, 


Causes,  52;  theories,  55;  symptoms,  58;  Laryngismus  stridulus,  59;  diag- 
nosis of  convulsions,  62;   treatment,  64. 


Si 


CHAPTER   III. 
Epilepsy, 67 

Symptoms  of  "grand  mal,"  68;  "petit  mal,"  70;  causes,  71;  diagnosis, 
74;  prognosis,  76;  pathological  anatomy,  77;  treatment,  80;  surgical 
treatment,  83. 

CHAPTER   IV. 
Hysteria 86 

Psychic  or  mental  hysteria,  87;  motor  manifestations,  89;  distinction  be- 
tween epilepsy  and  hystero-epilepsy,  92;  hysterical  paralysis,  94;  apho- 
nia, 95  ;  sensory  symptoms,  97  ;  visceral  hysteria,  100  ;  diagnosis  of 
hysteria,  102;  pathology,  103;  duration  and  courses,  103;  treatment, 
104. 


VI  COXTENTS. 


CHAPTER   V.  PAGE 

Chorea, 108 

Etiology,  1 08;  causes,  no;  symptoms,  112;  laryngeal  chorea,  114;  com- 
plications in  chorea,  116;  duration,  117;  diagnosis,  118;  morbid  anat- 
omy, 119;  prognosis,  123;  treatment.  124. 


CHAPTER  VI. 
Choreiform  Diseases, .  ...     129 

Hereditary  or  Huntington's  chorea,  129;  symptoms,  130;  pathological 
anatomy,  132;  treatment,  133;  Hereditary  chorea  without  dementia, 
133;  Habit  chorea,  134;  complex  Co-ordinated  movements  (complex 
tics),  134;  Gyrospasms  of  the  head,  135;  Chorea  electrica,  136;  Mala- 
die  des  tics  convulsifs,  137;  treatment,  138;  Thomsen's  disease,  139; 
Congenital  Paramyotonia,  141;    Paramyoclonus  multiplex,  141. 

CHAPTER  VII. 
Tetanus,      .        .  ...     143 

Etiology,  143;  symptoms,  145;  tetanus  neonatorum,  147;  pathological 
anatomy  and  morbid  pathology  of  tetanus,  149;  differential  diagnosis, 
152;  prognosis,  treatment,  153. 

CHAPTER  VIII. 

Tetany, *56 

Symptoms,  157;  etiology,  159;  symptoms  of  latent  period,  160;  differen- 
tial diagnosis,  161;  morbid  anatomy  and  pathology,  161;  treatment, 
163;  tetanoid  chorea,  164. 

CHAPTER  IX. 
Headaches,  . x66 

Classification,  166;  anaemic  headaches,  167;  neurasthenic  headaches,  169; 
headaches  due  to  transitory  hyperaemia,  170;  gastric  headaches,  171; 
headaches  due  to  genital  irritation,  171;  to  ear  disease,  171;  to  organic 
disease  of  the  brain,  172;  in  acute  infectious  diseases,  173;  malarial 
headaches,  173;  uraemic  headaches,  173;  toxic  headaches,  174;  those 
due  to  eye-strain,  174;  Migraine,  175;  etiology,  178;  pathology,  179; 
relation  to  eye-strain,  181;  diagnosis,  182;  prognosis,  182;  treatment, 
183. 

CHAPTER   X. 

The  Disorders  of  Sleep, J86 

Normal  sleep,  186;  causes  of  insomnia,  187;  Pavor  nocturnus,  188;  Enu- 
resis nocturna,  190;    Somnambulism,  191. 


CONTENTS.  VII 


CHAPTER  XI. 

PACE 

Vasomotor  and  Tropho-neuroses, 192 

Exophthalmic  goitre,  192;  symptoms,  192;  morbid  anatomy,  196;  treat- 
ment, 197;  Thyroidectomy,  198;  Thyroid  enlargement  at  the  age  of 
puberty,  199;  Tachycardia,  199;  Myxcedema,  199;  Angio-neurotic 
oedema,  201;   Raynaud's  disease,  203;   Facial  hemiatrophy,  204. 


PART    II. 

ORGANIC    DISEASES    OF    THE    NERVOUS    SYSTEM. 

CHAPTER   XII. 

Diseases  of  the  Peripheral  Nerves, 209 

Some  peripheral  palsies,  209;  brachial  plexus  lesions,  210;  Erb's,  210; 
Obstetrical  palsies,  210;  pathology,  211;  diagnosis,  212;  prognosis, 
213;  treatment,  214;  Paralysis  of  the  lower  limbs,  215;  Facial  palsy, 
216;  treatment,  223;  other  peripheral  palsies,  225;  Spasm,  Tic  convul- 
sif,  Wry  neck,  225. 

CHAPTER  XIII. 
Multiple  Neuritis, 227 

Symptoms,  227;  course,  232;  toxic  forms,  233;  toxaemic  forms,  234; 
malarial  neuritis,  234;  pathological  anatomy  of  multiple  neuritis,  235; 
diagnosis,  236;  treatment,  238;  Diphtheritic  paralysis,  240;  pathologi- 
cal anatomy,  243;  diagnosis,  244;  treatment,  248;  Lead  paralysis,  246; 
diagnosis  and  treatment,  248. 

DISEASES    OF    THE    SPINAL   CORD. 

CHAPTER  XIV. 

Infantile    Spinal    Paralysis  —  The    Essential    Paralysis    of    Chil- 
dren —  Poliomyelitis  Anterior  Acuta, 249 

Symptoms,  249;  distribution  of  paralysis,  252;  deformities,  256;  morbid 
anatomy  and  pathology,  258;  theory  of  the  disease,  263;  differential 
diagnosis,  264;  prognosis,  267;  treatment,  268;  Subacute  anterior 
poliomyelitis,  270. 


Vlll  CONTENTS. 


CHAPTER  XV. 

PAGE 

Acute  Myelitis,  ...  .  .  .        .     272 

Symptoms,  272;  origin  of  myelitis,  277;  pathology  and  morbid  anatomy, 
278;  differential  diagnosis,  282;  prognosis,  284;  treatment,  285;  in- 
juries of  the  spinal  cord,  288;   pathology,  290;   treatment,  291.   ■ 


CHAPTER  XVI. 

Syphilis  of  the  Spinal  Coed:      Specific  Myelitis  and  Meningo-mye- 
litis, 293 

Symptoms,  293;  Erb's  type,  294;  hereditary  syphilitic  affections,  296; 
differential  diagnosis,  297;  morbid  anatomy,  298;  prognosis,  301; 
treatment,  302. 

CHAPTER  XVII. 
Disseminated  Sclerosis, 304 

Symptoms,  304;  pathological  anatomy,  308;  atypical  forms  and  differen- 
tial diagnosis,  310;  paralysis  agitans  and  multiple  sclerosis,  311;  prog- 
nosis and  treatment,  313. 


CHAPTER  XVIII. 

Compression  of  the  Spinal  Cord  —  Pott's  Paralysis,  .        .        -315 

Causes  of  injury  to  the  cord  in  Pott's  disease,  315;  symptoms  of  Pott's 
paralysis,  317;  diagnosis,  318;  prognosis,  treatment,  319. 

CHAPTER    XIX. 

Tumors  of  the  Spinal  Cord  and  its  Meninges,     .        .        .        .  321 

Causes  and  symptoms,  321;  pathology,  diagnosis,  325;  prognosis,  treat- 
ment, 327;    Syringomyelia  and  gliosis  of  the  cord,  329. 

CHAPTER    XX. 

Family  Diseases  of  the  Spinal  or  Cerebro-Spinal  System,         .        .     ^^^ 

Hereditary  ataxy. — Friedreich's  disease,  333-334;  symptoms,  334;  differ- 
ential diagnosis,  339;  pathological  anatomy,  340;  Hereditary  ataxy 
(cerebellar  type;  type  Nonne-Marie),  344;  Hereditary  spastic  paraly- 
sis, 345;  spinal  type,  347;  diagnosis,  348;  cerebral  type,  348;  cerebral 
diplegia,  348;  morbid  anatomy  of  the  cerebral  type  of  hereditary  spas- 
tic paralysis,  349;  Little's  disease,  350. 


CONTENTS.  IX 


CHAPTER    XXI. 

PAGE 

Progressive  Muscular  Atrophies,  352 

Division  of  the  subject,  353;  distinction  between  progressive  amyotro- 
phies and  progressive  myopathies,  354;  type  Aran-Duchenne,  355;  He- 
reditary progressive  muscular  atrophy  (Hoffmann),  357;  Progressive 
neural  muscular  atrophy,  etc.,  359;  etiology,  diagnosis,  364;  pathol- 
ogy, 366;  Primary  myopathies,  368;  types  of  primary  dystrophies, 
369;  Muscular  pseudo-hypertrophy,  371;  Erb's  type,  or  the  juvenile- 
form,  374;  Landouzy-Dejerine  type,  374;  diagnosis  of  dystrophies, 
377;  physiological  hypertrophy,  377;  pathology,  379;  histological 
changes  in  muscles,  380;  duration,  treatment,  383;  Total  absence  and 
early  atrophy  of  muscles,  384. 

CHAPTER   XXII. 

Malformations  and  Conditions  due  to  Defective  Development  of 
the  Cord, 386 

Amyelia,  Atelomyelia,  Asymmetry,  Heterotopia,  386;  Diplomyelia,  387; 
Spina  bifida,  387;  symptoms,  389;  treatment,  390. 


DISEASES    OF    THE    BRAIN. 

CHAPTER   XXIII. 

Meningitis  and  Encephalitis,  ...  .  .    391 

Simple  acute  meningitis,  391;  etiology,  morbid  anatomy,  393;  diagnosis, 
394;  prognosis,  treatment,  395;  Tubercular  meningitis,  397;  onset, 
397;  symptoms,  398;  morbid  anatomy,  400;  pathology,  diagnosis, 
401;  prognosis,  402;  treatment,  403;  Epidemic  cerebro-spinal  menin- 
gitis, 404;  microbic  origin,  405;  history  of  epidemics,  405;  symptoms, 
407;  morbid  anatomy,  etc.,  409;  differential  diagnosis,  treatment,  410; 
Meningitis  due  to  traumatism  after  operation,  411;  Meningitis  due  to 
ear  disease,  412;  Meningitis  after  infectious  diseases,  414;  after  in- 
fluenza, 415;  Septicaemic  meningitis,  415;  Cnromc  basilar  meningitis, 
415;  Acute  encephalitis,  416;  Polio-encephalitis  superior,  417;  Bul- 
bar palsies,  418. 

CHAPTER  XXIV. 

Hydrocephalus, 42* 

Acute  hydrocephalus,  421;  meningitis  serosa,  422;  pathology  of  acute 
hydrocephalus,  422;  chronic  hydrocephalus,  423;  congenital  hydro- 
cephalus, 424;  acquired  internal  hydrocephalus,  426;  prognosis,  treat- 
ment, 428;  surgical  methods,  429. 


CONTENTS. 


CHAPTER    XXV. 


PAGE 


Infantile   Cerebral   Palsies    (Spastic   Hemiplegia,    Diplegia,   Para- 
plegia),  43° 

History  of  subject,  430;  frequency,  431;  distribution  of  paralysis,  433; 
onset,  434;  etiology,  435;  form  of  palsy,  438;  rigidities  and  contrac- 
tures, 440;  gait,  441;  post-paralytic  disturbances  of  motion,  441;  atro- 
phy of  muscles,  442;  epilepsy  with  cerebral  palsy,  444;  idiocy  with 
same,  444;  classification  of  palsies,  447;  morbid  anatomy,  446;  of 
congenital  cases,  447;  of  birth  palsies,  447;  of  acute  cerebral  palsies, 
449;  polio-encephalitis,  454;  differential  diagnosis,  455;  prognosis, 
456;  treatment,  457;  surgical  treatment,  460;  amaurotic  family  idiocy, 
462;  symptoms,  463;  diagnosis,  465 ;  pathological  anatomy,  465 ;  prog- 
nosis, 468;  treatment,  469. 


CHAPTER   XXVI. 

Tumors  of  the  Brain  and  its  Meninges,       ......     470 

Forms  of  tumor,  locations  of  tumors,  470;  etiology,  471;  symptoms,  . 
472;  Macewen's  symptom,  474;  tumors  of  cortex,  474;  of  frontal  lobe, 
of  third  frontal  convolution,  of  motor  area,  475;  of  parietal  lobe,  of  oc- 
cipital lobe,  of  temporo-sphenoidal  lobe,  476;  of  basal  ganglia,  of  crus 
cerebri,  of  the  corpora  quadrigemina,  477;  of  the  pons,  478;  of  the 
cerebellum,  479;  differential  diagnosis,  480;  pathology,  48 1 ;  treatment, 
484;  surgical  procedures,  486. 

CHAPTER   XXVII. 

Abscess  of  the  Brain,       . .    488 


Occurrence  of,  488;  connection  with  ear  disease,  489;  with  nasal  disease, 
490;  symptoms  of  abscess,  491;  differential  diagnosis,  493;  prognosis, 
494;  treatment,  495;  surgical  procedures,  495 ;  Thrombosis  of  the  in- 
tra-cranial  sinuses,  497;  symptoms,  498;  special  symptoms  of  throm- 
bosis, of  cavernous,  petrosal,  and  lateral  sinuses,  499;    treatment,  499. 


CHAPTER  XXVIII. 

Diseases  and    Conditions   due  to   Defective  Development   of  the 
Brain, •  501 

Larger  defects,  501;  Cyclops,  501;  Anencephalus,  Hemicephalus,  502; 
Porencephaly,  502;  congenital  porencephaly,  503;  acquired  poren- 
cephaly, 505;  Microcephalus,  506;  craniectomy,  Lannelongue's  pro- 
cedure, 509;  Partial  cerebral  defects,  511;  defective  development  of 
the  occipital  lobe,  511;  Agenesis  corticalis,  513;  Macrocephalus,  514; 
Defective  development  of  cranial  nerve  nuclei,  515;   Pleuroplegia,  517. 


CONTENTS.  X 1 


CHAPTER    XXIX. 

PAGE 

Insanity, 5 '9 

Differences  between  insanity  of  child  and  of  adult,  519;  frequency,  520; 
etiology,  520;  forms  of  insanity,  522;  Imperative  concepts,  522;  Mv- 
sophobia,  523;  Delire  du  toucher,  523;  Agoraphobia,  Cerebral  neu- 
rasthenia, 524;  Hypochondriasis,  526;  Mania,  527;  Melancholia,  529; 
Periodic  and  circular  insanity,  532;  Dementia  precox,  533;  Hebe- 
phrenia, 533;  Katatonia,  533;  Paranoia,  534;  Moral  insanity,  536;  Ep- 
ileptic insanity,  537;  Paretic  dementia,  538;  Masturbation  and  insan- 
ity, 530;  prognosis,  540;  treatment,  540. 


CHAPTER   XXX. 

Idiocy  and  Imbecility, 541 

Classification,  542;  hereditary  congenital  idiocy,  542;  developmental, 
acquired  idiocy,  543;  causes,  544;  symptoms,  546;  pathology,  551; 
prognosis,  treatment,  553;  Myxcedematous  idiocy — sporadic  cretinism, 
554;  diagnosis,  prognosis,  treatment,  556. 

APPENDIX. 

Localization  of  the  Functions  of  the  Segments  of  the  Spinal  Cord,     561 

Index, 563 


THE  NERVOUS  DISEASES  OF  CHILDREN. 

CHAPTER  I. 

INTRODUCTION— METHODS  OF  EXAMINATION. 

The  nervous  system  of  the  child  is  subject  to  many  dis- 
eases. Some  of  these  are  identical  with  the  nervous  dis- 
orders of  tne  adult ;  others  are  peculiar  to  the  early  years 
of  life. 

The  brain  and  the  spinal  cord  do  not  attain  their  full 
development  until  months  and  years  after  birth,  and  even 
the  peripheral  nerves  do  not  exhibit  all  their  normal  func- 
tions until  the  child  is  several  weeks  old.  During  this  pe- 
riod of  incomplete  development,  the  nervous  system  re- 
sponds much  more  readily  to  morbid  influences  than  it  does 
in  later  years.  This  is  especially  true  of  the  brain.  It  is  in 
a  state  of  irritability  and  instability,  and  a  perversion  of 
functions  may  result  from  causes  which  would  exert  little 
or  no  influence  over  the  nervous  system  of  the  youth  or 
adult.  Evidence  of  this  is  furnished  by  the  behavior  of  a 
chiid  in  fever.  The  irregular  choreiform  twitchings  and 
the  delirium  are  often  the  outward  signs  of  an  unstable 
cerebral  state,  while  the  unusual  irritability  of  the  brain  is 
proved  by  the  occurrence  of  convulsions  upon  peripheral 
irritation  to  which  the  adult  brain  would  not  at  all  respond. 

In  the  early  period  of  life,  too,  hereditary  affections  of 
the  nervous  system  are  frequently  manifest,  and  morbid 
psychic  inheritance  casts  its  shadows  before.  Inhibition  of 
normal  development  may  occur  at  any  period  ;  family  affec- 
tions are  developed  in  the  earlier  years  of  life,  and  the 
acute  infectious  diseases  of  childhood  are  often  followed  by 
serious  nervous  disorders.  If  we  add  to  these,  diseases  due 
to  traumatism,  we  have  an  array  of  nervous  disorders  equal 


2  THE  NERVOUS  DISEASES   OF  CHILDREN. 

to,  if  not  in  excess  of,  those  that  occur  after  the  age  of 
puberty.  Childhood  is  exempt  only  from  the  diseases  due 
to  senile  deterioration,  and  is  relatively  free  from  those  due 
to  toxic  agents,  such  as  alcohol,  metallic  poisons,  and  syph- 
ilis ;  but  the  effect  of  such  diseases  in  the  parent  is  exhib- 
ited with  cruel  persistence  in  the  offspring. 

The  diseases  of  the  nervous  system  during  the  period  of 
incomplete  development  are  to  be  the  special  subject  of 
this  treatise.  Before  proceeding  to  the  description  of  dis- 
ease it  will  be  necessary  to  adopt  a  correct 

Method  of  Examination. — First  of  all  inquire  into  the 
ancestry  of  the  child.  A  reliable  history  of  the  physical 
and  mental  condition  of  parents,  grandparents,  and  other 
relatives  is  of  the  greatest  value  in  establishing  a  diagnosis. 
The  habits  and  the  diseases  of  the  parents  should  be  care- 
fully determined,  for  of  the  ills  the  child  is  heir  to,  not  a  few 
are  due  to  alcoholism,  to  syphilis,  to  epilepsy,  to  hysteria, 
to  psychic  disorders  of  a  parent.  Next  to  heredity,  en- 
vironment plays  the  most  important  part;  it  is  well,  there- 
fore, to  inquire  into  the  home  surroundings,  the  manner  in 
which  the  child  is  watched  and  cared  for;  how  it  has  been 
fed,  trained,  and  educated. 

The  previous  history  of  the  child  is  next  in  order.  In 
every  case  inquire  into  the  manner  of  its  birth  ;  whether  it 
was  protracted  or  not ;  whether  or  not  instruments  were 
used ;  whether  the  child  was  asphyxiated  at  birth  or  at 
once  began  to  breathe  freely.  Make  inquiries  regarding 
the  occurrence  of  spasms  or  convulsions  ;  the  time  at  which 
the  child  began  to  take  notice  of  things,  to  recognize  parent 
or  nurse,  to  stand,  to  walk,  and  to  talk.  The  occurrence  of 
other  diseases,  of  the  ordinary  infectious  diseases  of  child- 
hood, of  whooping-cough,  of  pneumonia,  of  scarlet  fever, 
of  measles  and  meningitis,  should  be  determined,  and 
one  should  never  forget  to  ask  whether  similar  nervous 
conditions  have  been  previously  observed.  Then  proceed 
to  the 

Examination  of  the  Patient. — The  art  of  making  a 
diagnosis  by  mere  inspection  has  gone  out  of  date,  and  is 
cried  down  by  many;  yet  I  am  willing  to  say  that  in  fully 
one-half  of  the  nervous  diseases  of  children  the  nature  of 


INTRODUCTION — METHODS   OF  EXAMINATION.  3 

the  trouble  can  be  suspected,  if  not  made  out,  by  a  thor- 
ough inspection  of  the  child  without  putting  a  finger  to  its 
body.  I  am  not  in  favor  of  hurried  examinations  ;  on  the 
contrary,  I  wish  to  plead  for  the  greatest  accuracy  in  ex- 
amining for  details  ;  but  let  the  physician  or  student  train 
his  powers  of  observation  and  his  diagnostic  ability  will  be 
more  acutely  developed  than  that  of  the  man  who  can  never 
even  suspect  a  disease  unless  he  has  all  his  tools  (percus- 
sion-hammer, thermometer,  aesthesiometer,  electrodes)  con- 
stantly at  his  command. 

In  my  lectures  to  students  I  insist  that  they  shall  study  the  general  ap- 
pearance of  a  child,  and  should  not  feel  satisfied  until  they  learn  to  recognize 
peculiarities  of  facial  expression,  of  gait,  and  of  stature  ;  to  distinguish  be- 
tween the  behavior  of  the  normal  child  and  the  feeble-minded,  between 
spastic  and  flaccid  palsies,  and  to  determine  by  the  peculiar  deformity  of  the 
foot  or  by  the  scraping  noise  which  the  patient  makes  in  walking,  which 
group  of  muscles  is  affected.  It  is  important  from  the  history  of  the  patient, 
and  from  these  general  observations  to  get  correct  first  impressions ;  these 
first  impressions  are  then  to  be  confirmed  by  a  careful  detailed  examination. 

Never  make  a  diagnosis  unless  the  child  has  been  wholly 
undressed ;  if  this  is  not  done  a  Pott's  paralysis  may  be 
taken  to  be  a  traumatic  myelitis,  or  a  neuritis  may  be  mis- 
taken for  poliomyelitis  anterior.  Lay  the  child  on  a  table 
or  on  another  person's  lap  in  order  to  get  a  full  view  of  it ; 
of  the  relative  size  of  head  and  body ;  of  the  proportionate 
development  of  arms,  legs,  and  abdomen.  Remember  also 
that  the  child  has  heart  and  lungs,  liver,  spleen,  and  intes- 
tines, which,  if  diseased,  may  hold  an  important  relation  to 
the  nervous  disorder  present.  In  proceeding  to  a  detailed 
examination  it  is  best  to  begin  with  the  head,  including  the 
face,  then  take  up  the  upper  extremities,  the  abdomen,  and 
finally  the  lower  extremities. 

The  following  scheme  includes  the  more  important  points  to  be  estab- 
lished in  the  examination  of  a  child  ;  the  exact  order  of  inquiry  is  subject  to 
slight  modifications. 

EXAMINATION   SCHEME. 

Head  (Skull). — Size?  Shape?  Symmetrical?  Dolichocephalic?  Brachy- 
cephalic  ?  Fontanelles  ?  Hydrocephalus  ?  Bulging  (Frontal  or  occipital)  ? 
Position  of  head  ?     Stigmata  of   Degeneration  (such  as  prognathism,  de- 


4  THE   NERVOUS  DISEASES   OF   CHILDREN. 

formity  of  ears,  hair-lip)  ?  Pain  on  percussion  of  skull  ?  Mental  condition  ? 
Speech?     Sense  of  smell?  (use  peppermint). 

Eyes  (Fundus). — Vision?  Field  of  vision?  Pupils?  Light  and  ac- 
commodation reflexes  ?  Ocular  movements  ?  Nystagmus  (Lateral,  vertical,  or 
rotatory)  ?     Is  the  cornea  sensitive  ?     Photophobia  ?     Hearing  ? 

Face. — Symmetrical  ?  Paralysis  ?  Tongue  ?  Taste  ?  Deglutition  ? 
Articulation  ?     Sensation  in  face  ?     Teeth  ? 

Upper  Extremities. — Are  they  equally  developed  ?  Position?  Cir- 
cumference of  arm  and  forearm  ?  Movements  (of  shoulder,  arm,  forearm, 
wrist,  fingers)  ?  Paralysis  ?  Tonus  of  muscles  ?  Are  muscles  atrophied  or 
hypertrophied  ?    Reflexes  ?    Contractures  ?   Electrical  reactions  ?    Sensation  ? 

Spine. — Rigidity  ?  Lateral  curvature  ?  Kyphosis  ?  Lordosis  ?  Pain  on 
percussion  ? 

Trunk. — Respiration  ?  Sensation  ?  Reflexes  (Abdominal,  Epigastric, 
Cremasteric)  ?     Action  of  muscles  ? 

Lower  Extremities. — Are  they  symmetrical  ?  Circumference  of  thighs 
and  calves  ?  Ability  to  stand  ?  Romberg's  symptom  ?  Ability  to  walk  ? 
Gait  (Paretic,  Spastic-paretic,  Ataxic,  Cerebellar)  ?  Movements  of  individual 
groups  of  muscles  ?  Is  child  able  to  raise  thigh  ?  To  flex  and  extend 
thighs,  legs,  toes  ?  To  stand  on  tip-toes  ?  To  elevate  toes,  keeping  heels  on 
ground  ?  Are  muscles  paretic  or  paralyzed,  atrophied  or  hypertrophied  ? 
Electrical  reactions?  Reflexes  (Knee-jerks,  Ankle  clonus,  Babinski  reflex, 
plantar)  ?     Contractures  ?     Sensation  ? 

Vesical  and  Rectal  Reflexes? 

Examination  of  the  head  of  a  child  often  gives  us 
valuable  information.  The  normal  head  should  be  well 
rounded  and  symmetrical.  According  to  the  age  of  the 
child  the  size  will  vary.  The  average  horizontal  circum- 
ference at  birth  (measured  by  a  line  passing  from  the  gla- 
bella around  the  occipital  protuberance)  is  between  38  and 
42  cm. ;  at  the  end  of  one  year  between  45  and  52  cm.,  and 
in  later  years  it  may  grow  gradually  to  56  cm.  Any  marked 
departure  from  these  measurements  is  abnormal,  but  heads 
of  tolerable  size  may  be  associated  with  deficient  develop- 
ment of  parts  of  the  brain.  I  have  seen  cases  with  normal 
circumference  in  which  the  anterior  defect  was  not  evident 
in  the  measurement  in  consequence  of  a  slightly  excessive 
development  of  the  occiput.  A  normal  circumference  is 
also  present  at  times,  although  the  actual  cranial  capacity 
may  be  very  much  diminished  by  a  receding  frontal  bone. 

The  following  table  will  give  the  chief  measurements  of  the  skull  in  chil- 
dren ;  some  allowance  should  be  made  for  the  thickness  of  hair  and  scalp. 


INTRODUCTION— METHODS   OF  EXAMINATION.  5 

Table  of  Cranial  Measurements  in  Children. 


New-born. 

End  of  1st 

yr- 

1st  to  7th  yr.  10th  year. 

M. 

F. 

M. 

F. 

M.        F. 

M. 

F. 

47 

i.    Circumfer- 

2.   Binauri  c  u- 
lar  arc . . . 

34-o 

20.0 
385  to  450 

20.0 

7-7 
9.0 

34.o 
20.0 

42.0 

25- S 
700  to  1,000 

42.0 
25.0 

34  to  46 

27 

49 

Taken  around 
glabella    and 
occipital    pro- 
tuberance. 

Measured    from 

3.  Volume  . .. 

1,300 

12 

.... 

B  to   opposite 
ext.  aud.  mea- 
tus. 
Volume  is  to  cir- 

4. Naso -occi- 

pital arc. 

5.  Naso-breg- 

matic  arc. 

22.0 

7-7 
9.0 

28.0 

28.0 
10. 0 
10. 0 

cumference  as 
1,350  is  to  50 
(in  the  adult). 

N,  p,  T. 

N,  a. 

6  Bre  gmatic 
lamb,  arc. 

12 

a  to  A. 

Fig.  1. — Craniometrical  Lines.     (Benedict  and  Peterson.) 

The  formula  for  the  cephalic  index  is  length  :  breadth  ::  100  :  x.  An  in- 
dex below  78  is  dolichocephalic  ;  78  to  80  mesocephalic  ;  above  80  brachyce- 
phalic.  The  facial  length  is  determined  by  a  line  passing  from  N  to  lowest 
part  of  chin. 

Both  halves  of  the  head  should  be  symmetrical.    Asym- 
metry occurs  chiefly  in  connection  with  defective  develop- 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


Fig.  2. — Asymmetry  of  Skull  in  a  Male,  aged 
Six  Years.  Right  Hemiplegia  from  Birth. 
(Peterson.) 


ment  of  the  brain  and  with  early  cerebral  lesions.  (Fig. 
2.)  The  chief  abnormalities  of  skull  formation  are  as  fol- 
lows: Dolichocephalus,  a  long  skull,  the  anterior  poste- 
rior diameter  being  pro- 
portionately greater  than 
the  transverse.  Many  new- 
born children  are  dolicho- 
cephalic as  the  result  of 
compression  of  the  head 
in  the  pelvic  canal,  but  af- 
ter a  few  days  or  weeks 
the  head  is  well  rounded. 
Brachycephalic — the  skull 
is  short  in  the  antero-pos- 
terior  diameter.  The  terms 
microcephalus  and  macrocephalus  need  no  further  expla- 
nation. * 

Bulging  of  the  frontal  or  occipital  bones  is  important 
as  an  indication  of  hydrocephalus.  If  there  is  a  very  con- 
siderable increase  of  intracranial  fluid  the  sutures  may 
be  pushed  asunder  and  can  be  felt  distinctly  through  the 
scalp.  This  same  condition  occurs  in  some  cases  of  neo- 
plasm. In  passing  the  hand  over  the  head  the  fontanelles 
can  be  felt.  The  occipital  fontanelle  should  be  closed  after 
a  few  weeks,  the  anterior  remains  open  until  the  tenth  or 
twelfth  month.  If  it  closes  long  before  this  period  there  is 
premature  ossification  of  the  sutures  ;  if  it  remains  open 
much  longer,  it  is  a  probable  sign  of  rickets. 

i\fter  the  examination  of  the  head  we  may  pass  at  once 
to  the  inquiry  into  the  mental  condition  of  the  child.  Ac- 
cording to  its  age  we  must  ask  whether  it  recognizes 
its  mother  or  nurse;  whether  it  has  learned  to  play,  to 
understand  what  is  said  to  it ;  whether  it  begins  to  imi- 
tate sounds,  to  articulate,  etc. ;  in  short,  whether  it  shows  a 
normal  awakening  of  the  mind.  In  children  a  little  more 
advanced  in  years  it  is  necessary  to  determine  whether  the 
child  is  able  to  keep  up  with  others  of  its  age  ;  whether  it 

*  A  few  special  terms  have  been  in  use  for  oddly  shaped  heads  :  Keel-shaped  skull, 
scaphocephalus  ;  triangular  skull,  trigonocephalus  ;  steeple-shaped  skull,  oxycephalus ; 
obliquely  deformed  skull,  plagiocephalus. 


INTRODUCTION— -METHODS   OF  EXAMINATION.  J 

has  been  able  to  acquire  the  ordinary  rudiments  of  knowl- 
edge. The  physician  should  assure  himself  of  these  points 
by  a  personal  examination,  and  should  not  depend  upon  the 
statements  of  parents  and  relatives. 

A  few  extracts  from  Preyer's  observations  on  his  own 
child  will  show  what  may  be  expected  of  a  normal  child  at 
different  stages  of  its  development: 

During  First  Month.— Recognizes  difference  between  light  and 
dark  objects  (even  on  first  day)  ;  follows  with  its  eyes  object  moved  slowly 
before  it  (as  early  as  eleventh  day) ;  begins  to  hear  about  the  fourth  day ; 
recognizes  sounds  toward  end  of  first  month  ;  learns  to  distinguish  between 
bitter  and  sweet ;  recognizes  disagreeable  odors  ;  first  tears  on  twenty-third 
day  during  a  crying  spell ;  expresses  displeasure  by  turning  head  away,  by 
shutting  its  eyes,  and,  of  course,  by  crying  ;  begins  to  smile. 

During  Second  Month. — Recognizes  human  voices  and  direction 
from  which  sound  comes ;  turns  head  toward  low  sounds ;  is  quieted  by 
song ;  smiles  when  music  is  heard  ;  recognizes  its  mother. 

During  Third  Month. — Moves  arms,  expressive  of  pleasure  ;  listens 
attentively  ;  is  able  to  support  head  a  little  ;  uses  definite  sounds  in  crying. 

During  Fourth  Month. — Associated  eye  movements  perfect ;  stares 
at  new  objects  ;  recognizes  strange  surroundings  ;  reaches  after  distant  ob- 
jects ;  first  attempt  to  sit  upright. 

During  Fifth  Month.— Recognizes  strangers  as  such  ;  likes  to  take 
hold  of  everything ;  stretches  out  its  arms  to  be  taken  up  ;  holds  head 
straight ;  sits  alone  ;  moves  legs  as  if  to  walk  ;  forms  syllables. 

During  Sixth  Month.— Distinguishes  faces ;  stares  at  strangers ; 
smiles,  if  smiled  at ;  smiles  with  relatives,  not  with  strangers  ;  turns  its  head 
toward  a  person  leaving  the  room  ;  begins  to  creep  ;  "  crows." 

During  Seventh  Month.— Follows  objects  dropping  out  of  its 
hands ;  recognizes  its  image  in  mirror  with  evident  pleasure ;  points  with 
finger  at  pictures  ;  purposive  movements ;  associates  persons  and  names ; 
extends  hand  when  asked  ;  articulates  a  number  of  different  sounds  in  cry- 
ing and  in  "  lolling  to  itself." 

During  Eighth  Month.— Sits  upright  when  it  is  carried  ;  some  chil- 
dren attempt  to  stand  and  to  walk. 

During  Ninth  Month.— Begins  to  imitate  tunes  ;  laughs  heartily ; 
begins  to  beg  for  things. 

During  Tenth  Month.— Takes  an  intense  interest  in  its  food ;  rec- 
ognizes parent  after  absence  of  several  days  ;  he  begins  to  walk  alone  ;  an- 
swers questions  by  motions  and  indicates  where  certain  things  are. 

During  Eleventh  Month.— Stands  quite  alone;  pushes  chairs; 
makes  first  attempt  to  repeat  sounds  impressed  upon  its  mind ;  begins  to 
articulate  its  own  name  ;  understands  language  fairly  well. 

During  Twelfth  Month.— Imitates  laughter  of  others  ;  stretches  its 


8 


THE  NERVOUS  DISEASES   OF  CHILDREN. 


arms  out  to  enforce  its  demands  ;  improvement  in  walking  and  standing ; 
looks  at  others  attentively  while  they  eat. 

During  Fourteenth,  Fifteenth,  and  Sixteenth  Months. — In- 
dependent speech  is  acquired,  and  repeats  spoken  words  easily ;  in  seven- 
teenth month  may  speak  short  sentences,  using  verbs ;  from  this  time  on 
there  is  steady  improvement  in  memory  of  words  and  use  of  language. 

At  two  years  child  may  learn  to  repeat  rhymes,  to  detect  colors,  etc. 

If  the  mental  condition  of  the  child  has  been  satisfac- 
torily determined,  the  special  senses  should  be  examined. 
"  Does  the  child  see  ?  "  is  a  question  often  answered  affirma- 
tively by  the  mother,  when  a  closer  examination  proves 
that  the  child  is  totally  blind.  Mothers  are  easily  deceived 
in  this,  for  the  restless  movements  of  the  eyes  in  young 
children  are  supposed  to  be  purposive  and  part  of  the  vis- 
ual act.  To  test  vision  use  a  candle  or  a  taper,  and  pass  it 
in  front  of  the  eyes  at  some  distance  from  the  head,  so  as 
to  avoid  heat  sensations,  and  note  whether  the  child  fol- 
lows the  light.  Do  not  be  misled  by  accidental  movements 
and  avoid  using  rattles,  for  the  child  may  turn  its  eyes  in 


OD 


i"»«""" ""Green 

Fig.  3. — Normal  Visual  Fields  for  Colors. 


Fig.  4. — Normal  Visual  Field.     (Grasset.  et 
Rauzier. ) 


the  direction  from  which  the  sound  issues  without  seeing 
the  object.  Use  also  simple  substances  of  different  colors. 
A  blind  child  will  not  notice  an  approaching  finger  until 
the  eyelashes  have  been  touched. 

It  is  a  matter  of  still  greater  difficulty  to  test  the  field 


INTRODUCTION— METHODS   OF  EXAMINATION.  Q 

of  vision.  In  very  young  children  it  may  be  altogether 
impossible,  but  after  a  child  has  reached  the  age  of  five 
months  or  thereabouts,  it  may  be  possible  to  make  a  rough 
test  of  the  visual  field  by  passing  objects  from  above  and 
below,  as  well  as  from  the  sides,  into  the  visual  field,  and 
noticing  when  the  child  begins  to  turn  its  eyes  toward  this 


Fig.  5.— Field  of  Vision  in  a  Case  of  Left-sided  Hemianopsia.    The  Shading  Repre- 
sents the  Blind  Part;  the  Oval  Outline  is  the  Average  Normal  Field.     (Gowers.) 

object.  The  ordinary  test  as  applied  in  the  adult  cannot 
be  employed  for  children  until  they  have  developed  con- 
siderable intelligence. 

The  condition  of  the  visual  field  is  often  overlooked  and  the  defects  re- 
ferred to  are  much  more  frequent  than  they  are  generally  supposed  to  be. 
An  examination  should  be  made  in  all  cases  of  cerebral  palsies,  and  in  those 
in  which  cerebral  tumor  is  suspected. 

In  cases  in  which  a  very  complex  examination  seems  desirable,  and  these 
are  relatively  few,  special  tests  should  be  made  for  form  and  color.  The 
hemianopsic  defects  as  they  occur  in  tumors,  and  in  cerebral  palsies  (first 
described  by  Freud),  the  concentric  limitations  occurring  in  hysteria,  are 
the  most  common  defects  in  children. 

The  pupils  should  be  equal  and  contract  promptly  to  light  and  during  ac- 
commodation. They  should  also  contract  if  the  patient  attempts  to  close 
the  eyes  while  the  examiner  endeavors  to  keep  the  lids  open  (A.  Westphal 
and  Piltz). 

The  ocular  palsies  will  be  best  understood  by  reference  to 
the  subjoined  table.  Conjugate  deviation  is  due  to  disease 
in  the  hemisphere,  and  according  to  Grasset  to  lesions  in 
the  first  and  second  frontal  convolutions  and  in  the  angular 
gyrus  and  its  vicinity ;  the  patient  looks  toward  the  lesion 
except  in  the  case  of  spasm  of  the  muscles,  when  the  patient 
looks  away  from  the  lesion.  Nystagmus  occurs  in  many 
cerebral  affections,  particularly  in  cases  of  early  cerebral 
disease  or  congenital  defect.     It  is  seen  also  in  cases  in 


IO 


THE  NERVOUS  DISEASES   OF  CHILDREN. 


which  there  are  stigmata  of  degeneration  as  well  as  in 
multiple  sclerosis.  In  the  latter  it  may  be  bilateral  or 
rotatory.  The  corneal  reflexes  (the  prompt  closure  of  the 
eyelids  if  the  cornea  is  touched  carefully  with  the  head  of 
a  pin  or  a  small  lead-pencil)  should  be  tested.  In  a  great 
many  individuals  this  reflex  does  not  ensue  upon  irritation 
of  the  conjunctiva,  a  fact  that  has  deceived  many  physi- 
cians. 

Muscles  of  the  Eyes  and  Face. 


Diseases  in 

Name  of 

Normal 

Symptoms  of  Defi- 

Innervated 

Represented 

which  Muscle 

Muscle. 

Function. 

cient  Action. 

by 

in 

is  commonly 
Involved. 

Ciliary  . . . 

Makes  1  e  n  s  e 

Loss  of  accommo- 

The   third 

Corpora 

fa  «  J.  .J.  M 

|   rt  |  v  g'S 

more  convex ; 

dation  ;   spa_sm  of   nerve. 

quadrigem-i 

aids  in  accom- 

ciliary muscle  may 

ina  and  pe- 

3.2 c  ™  2 

modation. 

cause  myopia. 

duncle. 

«'°  o£  ° 

Sphincter  Contraction  of  Paralytic     mydria- The    third 

Corpora 

2  >^;3,°  a 

iridis. 

pupil  to  light 

sis ;     no    contrac- 

nerve. 

quadrigem- 

;^ft^ 

and     during 

tion   to   light  and 

ina  and  pe-. 

aumatisi 
eritic  pa 
;  tumor  ; 
:>r ;  in  c< 
iry  atax; 

accommoda- 

during conver- 

duncle. 

tion. 

gence   or   accom- 

modation. 

Dilatator  Dilates  pupils;'Pupil  does  not  re- Sympathetic 

~£g'C  .-a 

pupillse. 

as  a  result  of ,  spond  to  sensory 
sensory   or    stimulation. 

.is  "a  2  p.  "2  w 

psychic   stim- 

n.-S^I 

ulation. 

Meni 
eye 
ofm 
aliti 
lis; 
by  < 

Rectus  su- 

Moves eye  up- 

Upward movement  The    third 

Corpora 

f2S 

perior. 

ward  and  in- 

limited; diplopia; 

nerve. 

quadrigem- 

ward.      Acts 

false     image 

ina  and  pe- 

S3 

with    inferior 

above ;     deficient 

duncle. 

A  £2 

oblique. 

rotation     of   eye- 

■a & 

ball. 

Rectus  in- 

Moves  eye  in-'Strabismus    diver- 

As  above. 

Corpora 

.« s  ■ 

ternus. 

ward. 

gens;    defective 

quadrigem- 

rt  ua 

inward  pull. 

ina  and  pe- 
duncle. 

Rectus  in- 

Moves    eye  Imperfect  move- 

As  above. 

Corpora 

o  c  o 

ferior. 

downward 

ment    downward ; 

quadrigem-! 

S.V  g> 

and  rotates  it 

eye    rotated    out- 

ina and  pe- 

inward.   Acts 

ward. 

duncle. 

•-"5  ni 

with  superior 

u  c  v 
o  e~ 

oblique. 

B  ..s 

Obliquus 

Acts  with  rec- 

Tmperfect move- 

As above. 

C  o  r  p  o  r  a 

i  s  m  a 

inferior. 

tus    superior, 

ment  upward;  eye 

quadrigem- 

p-^.ti 

moving    eye 

rotated  inward. 

ina  and  pe- 

upward and 

duncle. 

outward,  and 

^  o 

rotates  it  up- 

C-   l_    0 

ward. 

Obliquus  M  ove  s    eye 

On  looking  down- 

The  fourth 

Peduncle 

"9  <u  _"d 

superior. 

downward 

ward  eye  is  pulled 

nerve    (tro- 

near   the 

and  outward. 

inward  ;      conver- 

chlearis). 

corpora 

Acts  with  in- 

gent  strabismus  ; 

quadrigem- 

P  -~     <~ 

ferior  rectus ; 

diplopia  on  step- 

ina. 

slc'-s 

rotates  down- 

ping downward. 

S'S    h 

ward. 

INTRODUCTION— METHODS   OF  EXAMINATION. 
Muscles  of  the  Eyes  and  Face. — Continued. 


I  I 


Diseases  in 

Name  of 

Normal 

Symptoms  of  Defi- 

Innervated 

Represented 

which  Muscle 

Muscle. 

Function. 

cient  Action. 

by 

in 

is  commonly 
Involved. 

Rectus  ex- 

Moves  eye  out- O  u  t  w  a  r  d   move- 

The    sixth 

As  before,  and 

ternus. 

ward. 

ment      impaired ;    nerve     (ab- 
head  turned  in  di-    ducens). 
rection    of    para- 
lyzed muscle. 

in  disease  of 
the  pons. 

Levator 

Raises     upper 

Ptosis  ;  eye  closed;  The  third  Peduncle 

Associated 

palpebrag 

eyelid. 

may  be  opened  a    nerve. 

and    cor- 

with    other 

superioris 

little   by  frontalis 

pora  quad- 

third  nerve 

muscle. 

rigemina. 

diseases;  often 
congenital. 

Orbicular- 

Closes eyelids. 

Peripheral    fa- 

is  palpe- 

closed   (lagoph-J  nerve. 

cial   neuritis  ; 

brarum. 

thalmos). 

affections  at 
base  of  brain 
(meningi  t  is  , 
tumor,  and 
the   like)  ;    in 

some    dystro- 

phies. 

Fronta  1  i  s 

Raises    eye- 

Imperfect    raising  As  above. 

As  above. 

As  above. 

and    cor- 

brows  ;     fold- 

of eyebrows ;    no] 

ruga  tor 

ing  of  skin  of 

frowning  ;  lines  of 

supercilii 

forehead,     as 
in  frowning. 

forehead   d  i  s  a  p- 
pear. 

Orbicula- 

Move lips  and 

Cerebral    apo- 

ris   oris, 

cheeks,   as  in 

pulled     toward    nerve. 

ple  xi  es  (es- 

buccin- 

speaking.kiss- 

healthy  side  ;  ina- 

cape of  upper 

ator,  and 

ing,    showing 

bility  to  pout  lips 

branches      of 

other 

of  teeth,   and 

or  to  whistle;  drib- 

fa c  i  a  1 )  ;  pe- 

muscl e  s 

the  like. 

bling  of   saliva 

ripheral  facial 

of  face. 

from     paralyzed 
side  ;    flapping  of 
cheek    with    each 
expiration,  owing 
to  paralysis  of 
buccinator  ;    d  i  s- 
app  earanceof 
nasolabial  fold. 

palsy  (often 
due  to  expos- 
u  re  )  ;  in  all 
lesions  of 
pons  ;  in  dys- 
trophies. 

Masseter , 

Masseter,  tem- 

Mastication imper- 

The   fifth 

Rarely    affect- 

tempo r- 

poral,  and  in- 

fect ;  if  in  spasm, 

nerve. 

ed  in  children 

al,    and 

ternal    ptery- 

jaws    cannot    be 

except  in  tet- 

ptery- 

goid    elevate 

opened;    jaw 

anus  ;  in  facial 

goids. 

lower   jaw  ; 

moved    toward 

hemiatrophy; 

external  pter- 

paralyzed side  by 

in    lesions 

ygoid     draws 

action     of   ptery- 

of pons. 

jaw  forward  ; 

goids. 

the  two  ptery- 

goid  muscles 

of    one    side 

acting  togeth- 

er move  teeth 

toward  oppo- 

site side. 

Mydriasis  can  be  caused  by  paralysis  of  sphincter  iridis  (third  nerve),  or 
by  spasm  of  the  dilator  muscles  (mydriasis  spastica).  Atropin,  Duboisin, 
and  Hyoscyamia  have  a  similar  effect  by  paralyzing  the  sphincter,  and  con- 


12  THE  NERVOUS  DISEASES   OF  CHILDREN. 

tracting  the  dilator.  Cocaine  produces  a  transitory  mydriasis  by  irritation  of 
the  pupillary  fibres  of  the  sympathetic. 

Myosis  may  be  due  to  irritation  of  the  sphincter  supplied  by  the  third 
nerve,  and  occurs  in  early  stages  of  many  cerebral  affections,  including 
apoplexy  ;  or  it  may  be  due  to  paralysis  of  the  dilator  muscle. 

Inequality  of  pupils  is  sometimes  congenital,  more  often  due,  however,  to 
cerebro-spinal  disease  (meningitis,  syphilis,  multiple  sclerosis,  hereditary  ataxia). 
Muscles  of  both  eyes  act  conjointly.  If  such  action  is  deficient  we  speak  of 
conjugated  paralysis.  There  may  be  paralysis  of  lateral,  upward  or  downward 
movements,  without  total  palsy  of  any  one  muscle.  If  individual  muscles 
are  paralyzed,  or  muscles  in  one  eye  only,  double  vision  is  certain  to  result. 

In  diplopia  the  exact  position  of  true  and  false  images  will  help  to  de- 
termine the  character  of  the  ocular  palsy.  If  one  muscle  is  paralyzed  second- 
ary deviation  of  the  associated  muscle  in  the  other  eye  may  take  place. 

Paralysis  of  the  inner  muscles  of  the  eye  is  designated  as  ophthalmo- 
plegia interna;  paralysis  of  the  muscles  moving  the  eyeball,  as  ophthalmo- 
plegia externa,  and  such  ophthalmoplegia  may  be  total  or  partial.  Total 
ophthalmoplegia  externa  and  interna  is  the  occasional  accompaniment  of  dis- 
ease at  the  base ;  partial  ophthalmoplegia  (only  part  of  the  muscles  sup- 
plied by  the  third  nerve,  for  instance,  being  affected)  points  to  nuclear  disease, 
but  syphilitic  infiltration  of  the  root  fibres  may  simulate  nuclear  disease. 

Narrowing  of  the  orbital  fissure  (sometimes  congenital)  may  be  due  to 
paralysis  of  smooth  fibres  in  the  lid,  innervated  by  the  sympathetic.  Clonic 
movements  of  eye  muscles  we  term  nystagmus ;  this  may  be  lateral,  vertical, 
or  rotatory. 

An  examination  of  the  sense  of  hearing  is  often  called 
for.  In  children  of  defective  development  this  sense  may 
be  entirely  wanting.  The  simplest  tests  are  to  clap  the 
hands  at  a  distance  from  the  child's  head,  to  whisper  its 
name,  to  use  a  loudly  ticking  watch — even  very  young 
children  will  be  attracted  by  the  sound  if  it  is  perceived. 
In  the  case  of  older  children  tuning-forks  maybe  applied  to 
the  head.  In  disease  of  the  ear  proper  the  vibrations  would 
be  perceived,  but  not  so  if  the  nerve  itself,  the  labyrinth,  or 
the  auditory  tract  were  involved.  Hyperacusis  occurs  in 
amaurotic  family  idiocy,  in  some  forms  of  meningitis. 

Asymmetry  of  the  face  may  point  to  congenital  defect 
or  to  a  preceding  palsy.  It  is  of  some  interest  to  know 
that  asymmetry  of  the  face  and  of  the  nose,  adhesion  of  the 
ear-lap,  malformations  of  the  outer  ear,  the  existence  of 
a  torus  palatinus  (felt  as  a  ridge  in  the  roof  of  the  mouth)  are 
the  ordinary  stigmata  of  a  degenerative  type,  of  which  so 
much  has  been  made  by  the  Italian  criminologist  of  the 


INTRODUCTION — METHODS   OF  EXAMINATION. 


13 


present  clay.  If  such  stigmata  prove  a  predisposition  to 
the  development  of  nervous  troubles,  to  crime,  or  to  in- 
sanity, it  is  well  that  the  physician  should  know  of  them. 

The  teeth  play  a  very  important  part  in  the  life  of  every  child.  If  not  de- 
veloped between  the  ages  of  six  months  and  one  year,  rickets  must  be  sus- 
pected. Notched  teeth  (second  dentition )  are  suspicious  of  syphilis.  Unusually 
early  development  of  the  teeth  is  an  evidence  of  premature  ossification. 

Muscles  of  Tongue,  Palate,  and  Pharynx. 


Name  of 
Muscle. 


Normal 
Function. 


Genio- 
glossus. 

Styloglos- 
sus. 


Lingual 
muscle 
proper. 


A  z  y  g  o  s 

uvulag. 


Le  vat  or 

palati. 


Palato- 
ph  ary  n- 
geal  mus- 
cles. 


Stylo-pha- 
ryngeus. 


Symptoms  of  Defi- 
cient Action. 


Pushes  tongue  Tongue  when  pro- 
to  opposite  truded  deviates  to 
side.  I  paralyzed  side. 

Raises  tongue  Tongue  cannot  be 
backward  moved  backward 
and  upward,  or  hollowed  out 
(action  deficient 
in  many  healthy 
subjects). 
All  movements  When    lying    in 


Innervated 
by 


Represented 


of  the  tongue 
itself. 


Shortening   of 
uvula. 


Raises  the  vel- 
um palati. 


Prevent     food 

from    passing!  food; 

toward  up-|  speech 

per  part    of 

pharynx    and 

posterior 

nares. 
Helps  to  draw 

larynx  up- 
ward so  as  to 

be   closed  by 

epiglottis  and 

ov  ertopp  e  d 

by  tongue. 


mouth  deviation 
to  healthy  side ; 
when  protruded 
deviates  to  paral- 
yzed side  ;  if  one 
or  both  halves  are 
atrophied  tongue 
looks  shrivelled. 

Uvula  deviates  to- 
ward sound  side  ; 
if  both  sides  are 
paralyzed  there  is 
nasal  tone  and 
regu  r  g  i  t  a  t  i  o  n 
through  nose. 

Arch  cannot  be 
raised  in  the  in- 
tonation of  "  ah ;  " 
if  paralysis  is  bi- 
lateral flapping  oi 
arch  and  regurgi- 
tation of  food 
through  nose. 

Regurgitation  of 
nasal 


The    twelfth  Medulla. 

nerve  (hy-1 

poglossal).  J 
The    twelfth  Medulla. 


The    twelfth  Medulla, 
nerve. 


Diseases  in 
which  Muscle  is 
commonly  In- 
volved. 


Probably 
pharyngeal 
plexus; 
seventh 
nerve  (?). 


As  above. 


Imperfect  degluti- 
tion ;  food  gets 
into  windpipe. 


Medulla . 


Medulla. 


The  fifth  Pons 
nerve. 


Glosso  -pha- 
ryngeal. 


Medulla . 


Bulbar  pal- 
sies (acute 
and  chron- 
ic) ;  in  spe- 
cific and  tu- 
fa e  r  c  ul  ar 
diseases  of 
base  ;  dys- 
rophies 
(rare). 


As  above 


As  above;  see 
also  seventh 
nerve  affec- 
tions. 


Basilar    affec- 
tions. 


Bulbar  affec- 
tions and  dis- 
eases of  the 
base. 


14 


THE  NERVOUS  DISEASES   OF   CHILDREN. 

Muscles  of  Tongue,  Palate,  and  Pharynx. — Continued. 


Diseases  in 

Name  of 

Normal 

Symptoms  of  Defi- 

Innervated 

Represented 

which  Muscle  is 

Muscle. 

Function. 

cient  Action. 

by 

in 

commonly  In- 
volved. 

Constri  c  - 

Help    to   push 

Food  is  swallowed 

P  h  a  ryngeal 

Medulla 

Diseases  of  the 

tors   of 

food  into  gul- 

very imperfectly  ; 

plexus. 

base  (bulbar). 

pharynx. 

let. 

sticks  in  throat. 

Laryngeal 

Movements  of 

Hoarseness    and  Recurrent 

Medulla .... 

Bulbar    troub- 

muscles. 

vocal  cords  in 

difficulty     in 

lar  yngeal 

les     (similar 

respirat  ion 

breathing ;   laryn- 

nerve  ex- 

sympto ms 

and  in  articu- 

goscopy examina- 

cepting the 

may  be  caused 

lation. 

tion  reveals  false 

crico- thy- 

by tumors  and 

position   of  vocal 

roid    mus- 

foreign bodies 

cords  (see  special 

cle. 

in  larynx). 

text-books). 

The  movements  of  the, tongue  have  been  considered  in  the  annexed  table. 
A  short  frenulum  may  cause  difficulty  in  articulation,  but  it  is  often  suspected 
to  be  the  cause  in  cases  in  which  the  defect  is  due  to  a  distinct  cerebral 
lesion. 

Sensory  disturbances  of  the  face  are  rare  in  children,  but,  if  suspected, 
tests  should  be  made  carefully  with  cotton,  with  the  head  and  point  of  a  pin, 

and  by  application  of  hot  and  cold  ob- 
r^^s^-  ^  jects.     Subjective  sensory  disturbances 

•&•-•  (neuralgia)  may  occur  as  in  adults,  and 

will  vary  in  distribution   according  to 
the  branches  affected.     (Fig.  6.) 

In  examining  the  trunk  and 
the  four  extremities  no  fact  is 
more    important    to    establish 
than  the  existence  of  paresis, 
or  paralysis  of  individual  mus- 
cles, or  of  groups  of  muscles. 
To  be  able  to  do  this  the  ex- 
aminer must  know  the  action 
of  all  the  more  important  mus- 
cles in  health  and  the  disturb- 
ances due  to   disease   of   such 
muscles.     These   are  the  very 
facts  in  which  the  student's  knowledge  is,  as  a  rule,  most 
defective.     In   the   appended    tables    I    shall   endeavor   to 
give  the  principal  points  in  concise  fashion  : 


Fig.  6. — Sensory  Chart  of  Face. 
(C.  S.  Freund.) 


INTRODUCTION— METHODS   OF  EXAMINATION. 


15 


Muscles  of  Head  and  Neck. 


Name  of 
Muscle. 


Normal 
Function. 


Sterno-Raises  and 
c  1  e  i  d  o-  turns  face  to 
mastoid,  opposite  side 
head  inclines 
to  same  side ; 
if  both  mus- 
cles act  con- 
jointly head  is 
brought  for- 
ward. 


R  e  c  t  u  s  To  flex  head, 
capitis 
anticus 
major. 


R  e  c  t  u  s  To  flex  head, 
capitis 
anticus 
minor. 

Rectus  Slight    rota 
capi  ti  s 
lateralis. 


Symptoms  of  Defi- 
cient Action. 


Inability    to    raise 
head  from  bed,  or 

'  other  horizontal 
position,  if  both 
muscles  are  affect 
ed  ;  if  one  muscle 
is  affected,  no 
marked  change  of 
position,  unless 
opposite  muscle 
is  contractured ; 
spasm  of  muscle 
frequent ;  head  in- 
clined to  one  side. 

Cannot  flex  head  ] 
so  as    to  bring 
chin  on  chest. 


Innervated       Represented 
by 


Diseases  in 
which  Muscle  is 
commonly  In- 
volved. 


Scalenil 
anterior 
med  i- 
u  s  ,  e  t 
po  s  t  e- 
rior. 


L  o  ngus 
colli. 


Elevate  ribs 
when  verte- 
bral column 
is  fixed ;  aid 
in  i  n  s  p  i  r  a- 
tion ;  slight 
lateral  flex- 
ion. 

Flexor  of  ver- 
t  e  b  r  a  1  col- 
umn. 


Deficient  rota- 
tion scarcely 
noticeable,  un- 
less sterno-clei- 
do-mastoids  are 
diseased. 

Deficient  inspi- 
ratory move- 
ments. 


Imperfect  flexion 
of  upper  spine,  j 


Spinal  ac- 
cessory. 


Upper  cervi 
cal. 


Medulla  and  In  bulbar  and 


affections  ;  in 
later  stages  of 
pro  gressive 
muscular 
a  t  r  o  p  h  i  es  ; 
o  c  c  asionally 
in  neuritis. 


Lower  cervi- 
cal nerves. 


Lower  cervi- 
cal nerves. 


second 
third 
vical 
ments. 

and 

cer- 
seg- 

Upper 
vical 
ments. 

cer- 
seg- 

Lower  cervi- 

c  a  1  s 
ments. 

eg- 

Diseases  of 
the  cervical 
region  (my- 
elitis, men- 
\  i  n  g  i  t  i  s, 
tumor ;  pro- 
gressive 
wasting  of 
muscles. 


The  sterno-cleido-mastoid  and  the  other  muscles  of  the  head  and  neck, 
mentioned  in  these  two  tables,  are  more  frequently  the  seat  of  spasm  than  of 
paralysis.  The  spasm  may  be  restricted  to  one  muscle  or  may  involve 
several.  It  may  be  the  result  of  organic  disease  in  the  medulla  oblongata 
or  spinal  cord.  More  often  it  is  functional  in  character  and  related  to  other 
spasmodic  conditions.  The  same  group  of  muscles  may  be  involved  in  the 
ordinary  rheumatic  affections  (torticollis,  caput  obstipum). 


i6 


THE  NERVOUS  DISEASES   OF  CHILDREN. 
Muscles  of  Shoulders  and  Upper  Extremity. 


Name  of 
Muscle. 


Trapezius, 
i.  Cla  - 
v  i  c  u  1  ar 
portion 
(respi- 
ratory; 
outer 
third  ot 
c  la  v  icle 
to  occipi- 
tal bone). 


Normal 
Function. 


Pulls  head 
backward ;  ro- 
tates slightly 
toward  side  ot 
muscle,  so 
that  chin  is 
turned  to  op- 
posite side  ; 
contraction  ot 
both  clavicu- 
lar portions, 
bends  head 
backward; 
slight  eleva- 
tion of  shoul- 
ders ;  aids  in 
deep  inspira- 
tion. 

Raises  shoul- 
der -  blade  ; 
elevation  of 
acromion 
(clavicle  goes 
along). 


Symptoms  of  Defi- 
cient Action. 


2.  Middle 
portion 
(from 
acromion 
and  outer 
spine  o  f 
scapula  to 
ligament, 
n  u  c  h  se 
and  up- 
per dorsal 
spines). 


3.   Low  e  r  Adduction     ot 
portion,  scapula  to- 


and     ad- 
ductor. 


R  h  o  m 

boids. 


ward   median 
line. 


Oblique  move 
ment  of  scap 
ula  from  be 
low,  upward 
and  inward 
so  that  infe- 
rior angle  is 
brought  near- 
er the  medi- 
an line  ;  hold 
spinal  margin 
of  scapula 
down  to  tho- 
rax. 


Deficient  back- 
ward movement 
of  head;  not 
marked  as  rule 
because  deep 
muscles  perform 
this  function  ; 
shoulder  does  not 
move  during  in- 
spiration. 


Innervated 
by 


Spinal  ac- 
cessory 
nerve. 


Acromion  depress- 
ed by  weight  ot 
upper  extremity  ; 
inner  upper  an- 
gle may  be  pulled 
upward  by  levator 
anguli  scapulas ; 
internal  lower  an- 
gle is  nearer  to 
median  line. 


Margin  of  scapula 
is  about  ten  ctm. 
distant,  instead  of 
being  five  or  six 
ctm.  distant  from 
median  line  ;  loss 
of  adductor  may 
be  covered  up  by 
action  of  rhom- 
boids ;  rounding 
of  back.    (Fig.  8.) 


Deep  groove  be-  Fifth  cervi- 
tween  inner  mar-  cal 
gin  of  scapula  and 
thorax  ;  if  serra- 
tus  is  normal,  this 
groove  disap- 
pears if  arm  is  ex- 
tended forward  ; 
shoulder  -blade 
cannot  be  approx- 
imated to  median 
line.  (According 
to  Duchenne  this 
can  be  affected  by 
upper  portion  of 
latissimus  dorsi.)   I 


Spinal      ac- 
cessory. 


Represented 


Medulla 
and  second 
and  third 
cervical 
segments 


As  above. 


Spinal      ac-J  Medulla 
c  e  s  s  o  r  y    and  second 
and    third 
cervical 
segments. 


Fourth  and 
Fifth  cervi- 
c  al  seg- 
ments, 


Diseases  in 
which  Muscle  is 
commonly  In- 
volved. 


P  r  o  g  r  e  ssive 
muscular 
wasting  ;  dis- 
eases of  me- 
dulla and  up- 
per cervical 
cord;  clavi- 
cular portion 
least  fre- 
quently in- 
volved. 


As  above. 


As  above. 


INTRODUCTION — METHODS    OF   EXAM/NATION. 


17 


Frontalis 

Facial  Nerve 

Corrugator  Supercilii 

Orbicular. 
Palpebr. 

Muscles  of  Noso 

Zygomatici 

Orbicularis  Oris 

Median  Division  of  1 

Facial  Nerve        f 

JVIasseter 

Levator  Menti 

Hypoglossal  Nerve 

Lower  Facial  Nerve 

Platysma  Myoides 

Hyoid  Muscles 

Omohyoid 


Anterior  Thoracic  Sup- ) 
plying  Pectoral  Mus- 
cles I 


Phrenic  Nerve        Erb's  Point  Brachial  Plexus 

Fig.  7. — Motor  Points  of  Face.     (Erb.) 


I  Region  of  Central 
(      Convolutions 


Speech  Area 
Temporal 

Facial 

Trunk  of  Facial  Nerve 

J  Posterior  Auricular 

I      Nerve 

Facial 

Lower  FJranch 
Splenius 

Sternocleido  Mastoid 
Spinal  Accessory  Nerve 
Levator  Angult  Scapulae 

Trapezius 
Scapular  Branch 

Circumflex  Nerve 
PosteriorThoracicNerre 


Fig.  8. — Patient  with  Hypertrophy  of  Infra  and  Supra  Spinati,  Showing  Rotation  of 
Right  Scapula  and  Deep  Groove  along  Inner  Margin  of  Scapula  (Atrophy  of 
Rhomboids  and  Slight  Atrophy  of  Lower  Portion  of  Trapezius). 


1 8  THE  NERVOUS  DISEASES   OF   CHILDREN. 

Muscles  of  Shoulders  and  Upper  Extremity. — Continued. 


Name  of 
Muscle. 


Levator 
anguli 
scapulae. 


Serr  a  t  u  s 
magnus. 


Deltoid 
(three    di- 
visions). 


Infraspi-  "I 
natus.     I 

Teres 
minor. 

Sub  scap- 
ularis. 


S  u  praspi- 
natus. 


Normal 
Function. 


Symptoms  of  Defi- 
cient Action. 


Draws  superi- 
or inner  angle 
of  scapula  up- 
ward :  aids 
in  shrugging 
of  shoulders. 

Rotation  o  t 
shoulder- 
blade  out-, 
ward,  and 
slight  eleva- 
tion of  acro- 
mion ;  holds 
inner  margin1 
of  scapula  to 
thorax;  brings 
arm  from  hor- 
izontal to  ver- 
tical position. 


Isolated    paralysis 
rare. 


To  raise  arm 
to  horizontal 
position,  and 
forward,  out-j 
ward,  or  back- 
ward ;  move- 
ments possi- 
ble only  if. 
scapula  is 
fixed  by  ac- 
tion of  serra- 
tus  and  tra- 
pezius. 

Rotator      hu- 
meri posticus 
(Duchenne)  ; 
rotate  arm; 
outward. 

Rotator  hume- 
ri an  ticus 
(Duchenne)  ;  j 
rotates  arm 
inward. 

Helps  to 
steady  shoul-j 
der-joint  and 
to  elevate 
arm  forward  I 
and  outward  ; 
outer  angle  of 
scapula  is  de-| 
pressed. 


Scapula  pulled  up 
ward  ;  lower  inner 
angle  nearer  the 
median  line  ;  arm 
cannot  be  raised 
above  horizontal 
position  ;  if  arm  is 
stretched  forward 
scapula  is  re- 
moved from  tho- 
rax ("winged 
scapula  ") ;  dur- 
ing abduction  of 
arm,  scapula  is 
moved  nearer  to 
median  line,  and 
crowds  trapezius 
and  rhomboids 
forward. 

Can  raise  shoulder 
but  not  arm; 
shoulder  flattened 
(atrophy)  ;  groove 
between  acromion 
and  head  of  hu- 
merus ;  each  di- 
vision of  deltoid 
may  be  paralyzed 
singly. 


Innervated 
by 


Third  and 
Fifth  cervi- 
cal nerves. 


Posterior 
thoracic 
nerve. 


Arm      cannot     be 
moved  outward. 


Circumflex. 


Supra  -1 
scapular.   I 

Difficulty  in    writ-  CLi  r  c  u  m  "  | 

ing  (Duchenne).         eX-  J. 

Arm     cannot      be  Subscapu-  j 
moved  inward  ;|  lar  nerve, 
scapula  is  rubbed 
against  ribs. 


According  to  Du- 
chenne, humerus 
is  separated  still 
further  from  acro- 
mion, if  supra- 
spinatus  is  affect- 
ed in  addition  to 
deltoid. 


Sup  rascap- 
ular. 


Represented 


Second  and 
Fourth  (?) 
c  er vical 
segments 


Fifth  and 
sixth  cervi 
c  al  seg- 
ments. 


Fourth,  fifth, 
and  sixth 
cervical 
segments, 


Fourth,  fifth, 
and  sixth 
cervical 
segments, 


Fourth 
vical. 


Diseases  in 
which  Muscle  is 
commonly  In- 
volved. 


Dys  t  rophies 
and  cervical 
diseases. 


P  r  ogressive 
muscular  at- 
rophies (dys- 
trophies); 
ne  u  r  i  t  i  s  of 
part  of  the 
brachial  plex- 
us; after  trau- 
matic injuries 
to  shoulder  ; 
i  n  cervical 
cord  affec- 
tions. 


As  above;  also 
in  Erb's  form 
of  obstetrical 
paralysis. 


As  in  case  of 

deltoid. 


As  above. 


INTRODUCTION— METHODS   OF  EXAMINATION. 


19 


20  THE  NERVOUS  DISEASES   OF   CHILDREN; 

Muscles  of  Shoulders  and  Upper  Extremity. — Continued. 


Diseases  in 

Name  of 

Normal 

Symptoms  of  Defi- 

Innervated      Represented 

which  Muscle  is 

Muscle. 

Function. 

cient  Action. 

by                       in 

commonly  In- 
volved. 

La  ti  ssi- 

Pulls  the  arm, 

Arm      cannot      be 

S  ub  scapu-,S  ix  th  and 

As  in  progres- 

mus  dor- 

when    raised, 

moved  backward  ; 

lar,    also'  seventh cer- 

sive  atrophies 

si. 

downward 

insufficient  exten- 

branches of 

vical. 

and     dystro- 

and back- 

sion     of      dorsal 

dorsal    and 

phies  ;  in  cer- 

ward  ;  if  arm 

spine  ;   trunk  can- 

lumbar 

vico  -dorsal 

is  at  rest  up- 

not  be  moved  lat- 

nerves pass- 

lesions;   in 

p  e  r     portion 

erally. 

ing  through 

neuritis. 

brings     scap- 

muscle. 

u  1  a      nearer 

the     median 

line ;      united 

action  of  up- 

per   third    of 

both   muscles 

causes  exten- 

sion of  dorsal 

trunk  ;   single 

action  causes 

lateral  move- 

mentof  trunk. 

Teres  ma- 

Rotates raised 

Very     few     symp- 

S u  b  s  c  apu- 

Seventh  cer- 

As above. 

jor. 

humerus      in- 
ward ;  adduc- 
tion of  arm  to 
thorax  ;  slight 
elevation      of 
shoulder. 

toms  ;  action  sup- 
plied     by     other 
muscles. 

lar. 

vical. 

Pectoralis 

Clavicular 

Imperfect     adduc- 

Anterior 

Fifth,    sixth, 

Amyotrophies 

major. 

portion     de- 

tion of  arm  ;   par- 

thoracic. 

and  seventh 

and      dystro- 

presses      hu- 

alysis can  be  dis- 

cervical. 

phies,  chiefly; 

merus      from 

covered    best    by 

also  in  lesions 

raised      posi- 

extending   arms 

of  brachial 

tion    to   hori- 

and tryingto  press 

plexus. 

zontal  ;       ad- 

volar     surfaces 

du  c  t  i  o  n  of 

against    each 

arm,      as      in 

other. 

giving  a  bless- 

ing ;     sternal 

p  o  r  t  i  on  de- 

presses    arm 

c  o  m  p  letely, 

and  if  arm  is 

at  rest  draws 

acromion  for- 

w  a  r  d    and 

backward. 

Absolute  paralysis  of  both  upper  extremities  is  rare  in  children.  If  an 
entire  extremity  is  paralyzed,  the  arm  either  hangs  limp  by  the  side  of  the 
body,  or  is  flexed  at  the  elbow.  If  the  arm  is  lifted  it  falls  by  its  own  weight. 
Children  sometimes  refuse  to  make  an  effort,  or  do  not  understand  what  is 
wanted  of  them.  After  a  few  trials  it  is  generally  possible  to  decide  whether 
there  is  a  loss  of  power  or  mere  lack  of  effort.  Total  paralysis  of  the  upper 
extremity  occurs  either  from  disease  of  the  brachial  plexus,  from  spinal  cord 
affections,  or  from  cerebral  lesions.     If  due  to  the  first  or  second  causes,  the 


INTRODUCTION — METHODS   OF   EXAMINATION. 


21 


Fig.  II. — Young  Boy  with  Multiple  Neuritis,  showing  Double  "Wrist  Drop"  and 

Slight  "  Foot  Drop." 


FlG.   12. — Appearance  of  Hand  in  an  Early  Stage  of  Progressive  Muscular  Atrophy; 
Atrophy  of  Abductor  Brevis  and  Opponens  Pollicis.     (Duchenne. ) 


22 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


paralysis  is  flaccid ;  if  due  to  the  third  cause,  the  paralysis  is  spastic,  and 
very  often  part  of  a  hemiplegia.  Plexus  lesions  are  observed  in  very  young 
children  (obstetrical  palsies),  spinal  lesions  in  children  of  any  age,  and  cere- 
bral lesions  in  children  under  the  age  of  four  chiefly  ;  but  the  after-effects  of 
these  conditions  are  often  visible  late  in  life.  From  the  causes  enumerated 
above,  as  well  as  in  cases  of  neuritis  and  progressive  muscular  diseases,  the 
paralysis  may  be  incomplete  and  restricted  to  definite  groups  of  muscles  only. 
To  test  the  extent  of  paralysis,  the  patient  should  be  asked  to  perform  the 
various  movements,  such  as  raising  the  shoulder,  putting  out  arm,  flexing 
and  extending  the  elbow,  wrist,  and  fingers.  In  very  young  children  these 
tests  cannot  always  be  satisfactorily  made,  but  even  in  infants  much  can  be 
inferred  from  their  power  to  hold  or  to  grasp  objects  placed  in  front,  above, 
or  below  them.  In  older  children  further  tests  can  be  made  by  offering  re- 
sistance to  active  movements,  and  asking  them  to  overcome  it.  The  ability 
to  bring  the  fingers  in  opposition  to  one  another,  to  make  the  thumb  touch 
the  tips  of  the  other  fingers,  the  moving  of  the  fingers  to  and  from  the  middle 
one,  and  the  ability  to  write,  are  necessary  tests  of  the  action  of  the  intrin- 
sic muscles  of  the  hand. 

Muscles  of  Arm,  Forearm,  and  Hand. 


TV 

Diseases  in 

Name  of 

Normal 

Symptoms  of  Defi- 

Innervated 

Represented    which  Muscle   is 

Muscle. 

Function. 

cient  Action. 

by 

in                  commonly  In- 

volved. 

Triceps. . . 

Extends  fore- 

Arm cannot  be  ex-  M  usculo- 

Six  th,   s  e  v- 

•  Poliomyelitis 

arm  ;  long1  tended  except  by    spiral. 

enth,  eighth 

and  other 

head  of  t  r  i-l  its  own  weight ;  if 

cervi  cal 

affections  of 

ceps,  and  cor-    long   head  of  tri- 

segments. 

c  er v  i  c  a  1 

aco-brachialis;  ceps   is  affected 

cord  ;  trau- 

help to  keep    subluxa  t  i  o  n   of 

matic    inju- 

head  of  hu- 

head of  humerus 

ries  ;  amyo- 

merus  in  posi- 

occurs easily. 

.    trophies 

tion. 

|     and  dystro- 

Biceps.... 

Flexion  ah  d 

Flexion    deficient,!  Musculo-cu- 

Fourth,  fifth, 

phies    (t  r  i- 

supination   of 

but  can  be  carried    taneous. 

sixth  cervi-. 

ceps   es- 

forearm. 

out   in   part    by 
other  muscles. 

cal. 

capes   in 
many  pe- 
1    r  i  p  h  e  r  a  1 
j     palsies). 

Supinato  r 

Flex e s  fore- 

Flexion and  prona-  Musculo- 

Fourth,  fifth1 

As  above  ;  in- 

longus 

arm  and  aids 

tion   deficient;    spiral. 

cervical. 

volved  in  pe- 

in pronation. 

muscle    does   not 
stand  out  pr  o  mi-j 
nently   if  arm  is 

ripheral  neu- 
ritis (trauma- 
t  i  c),  not   in 

flexed   and   at- 

lead palsy. 

tempt  is  made  by 

another  to  extend 

it  forcibly;  if  mus- 

cle   is    atrophied 

arm   is   spin  d  1  e- 

shaped 

Supinato  r 

Supinates 

Deficient     supina- 

Musculo- 

Fifth   cer- 

Dise a  s  e  s  as 

brevis. 

han  d   when 
forearm  is  ex- 
tended. 

tion  of  hand. 

spiral. 

vical. 

above  ;  also 
in  peripheral 
palsies. 

INTRODUCTION— METHODS   OF  EXAMINATION. 


23 


Fig.  13. — Motor  Points  on  Shoulder  and  Arm.     (Bernhardt.) 


Supinator  Longus 
Extensor  Rad.  Long- 
Extensor  Rad.  Brev.- 
Extensor  Digitorum  Communis 
Extensor  Indicis' 

Abductor  Pollicis  Longu: 
Extensor  Pollicis  Brevi! 


Dorsal  Interosseil.  and  II.  | 


Extensor  Carpi  Ulnaris 
Supinator  Brevis 


Extensor  Minimi  Digiti 
"Extensor  Indicis 


Extensor  Pollicis  Longus 


Abductor  Minimi  Digiti 
Dorsal  Interossei  III.  and  IV. 


Fig.  14. — Motor  Points  of  Forearm  and  Hand.     (Erb.) 


24 


THE   NERVOUS  DISEASES   OF   CHILDREN. 
Muscles  of  Arm,  Forearm,  and  Hand. — Continued. 


Diseases  in 

which  Muscle  is 

Name  of 

Normal 

Symptoms  of  Defi- 

Innervated 

Represented 

commonly  In- 

Muscle. 

Function. 

cient  Action. 

by 

in 

volved. 

Exten  s  o  r 

Ex  tens  ion 

Wrist    cann  o  t  be 

M  usculo- 

Seventh  cer- 

As before  ;  es- 

carpi 

and   abduc- 

flexed d  o  r  s  a  lly 

spiral. 

vical. 

pecially  in 

rad  i  a  1  i  s 

tion  of  wrist ; 

(extended)  or  ab- 

neuritis. 

longus  et 

the   sh  o  r  t  e  r 

ducted  ;  flattening 

brevis. 

muse  le  has 
pure   ex  ten- 
sion action 

of  forearm.     (Fig. 
ii.) 

1  only. 

Ex  t  e  n- Extension   and 

Wrist    canno  t    be 

As  above.       Seventh  cer- 

As  above. 

sor  carp  i    abduction    ot 

flexed  dorsally  or 

vical. 

ulnaris. 

wrist. 

adducted  ;  "  drop 
wrist  "   is  charac- 
teristic of  paraly- 

sis of  extensors. 

Exte  n  s  o  r 

' 

First   pha  langes 

Musculo-  Seventh  cer- 

As  above. 

d  i  g  i  t  o- 

cannot    be    ex- 

spiral. 

vical. 

rum  com- 

Extension of 

tended  nor  fingers 

munis. 

first    pha- 

abducted  ;    grasp 

Exte  n  s  o  ri  \   langes  of  all 

is   weak   b  ecause 

indicis.        j    fingers  and 

flexor  muscles  are 

Extensor'      abduction. 

sho  r  t  e  n  e  d  and 

minimi!  | 

cannot   contr  act 

digiti.         J 

forcibly. 

F  lexorFlexion    of 

Deficient  flexion. 

Eighth    cer- 

As above. 

carpi    wrist  and  pro- 

vical. 

radialis.       nation. 

Flexor!  Flex  ion    of 

Flexion  and   supi- 

Ulnar  

Eighth    cer- 

As above. 

carpi 

wrist    and 

nation  impaired. 

vical. 

ulnaris. 

supination. 

Pal  maris 

Flexion    of 

Flexion   impaired ; 

Eighth    cer- 

As above. 

longus. 

wrist  only. 

no   anomalous 
position   of  hand 
from  paralysis   of 
wrist  as  hand  falls 
by  its  own  weight; 
the   flexors   of 
fingers  may  act  as 
substitutes. 

vical. 

F  1  e  x  o  r 

Flexes  second  Second   phalanx 

Eighth    cer- 

As above. 

d  i  g  i  t  o- 

phal  a  n  x   to- 

cannot  be  flexed. 

vical. 

rum  sub-;  ward  first. 

limis. 

F  1  e  x  o  r 

Flexes     last 

Last  two  phalanges 

Ulnar    and 

Eighth    cer- 

As    above; 

d  igi  to- 

two  phalanges 

cannot  be  flexed. 

Median. 

vical. 

muscle  should 

rum  pr  o- 

toward  first. 

be  tested  with 

fundus. 

special    c  a  re 
in    ca  s  e  s    of 
traumatic   in- 

juries. 

Interossei  Abduction  and  Fingers  cannot  be 

Ulnar,  which 

Eighth    cer- 

As above  ;  o  f- 

and  lum- 

adduction    of 

abducte  d   or   ad- 

also   sup- 

vical,  fir  s  t 

ten    the  first 

bricales. 

fingers  if  first 

duc  t  e  d  ;    inte  r- 

plies  th  i  r  d 

dorsal. 

muscles  to  be 

phalanges  are 

osseous    spaces 

and    fourth 

affected    in 

extended; 

are  very  marked  ; 

lu  m  b  rica- 

progre  s  s  i  v  e 

flexion  of  first 

"  Main  en  griffe  " 

les;  median 

spinal   atro- 

phalanges 

due  to   extension 

supplies 

phies. 

and    s  i  m  u  1- 

of  first  phalanges 

first  two  and 

tane  o  u  s   ex- 

and  flexion    of 

somet  i  m  es 

tension  of  sec-1 

second    and  third 

third   1  u  m- 

ond  and  third    phalanges.   (Figs. 

bricales. 

phalanges. 

i7l  18.) 

INTRODUCTION— ME  THODS   O/-    EXAMINATION. 


25 


Triceps  (External  Ilea 

Triceps  (Internal  Hea 

Ulnar  Ner 


Flexor  Carpi  Uli 
Flexor  Profundus  Digitorun 


Flexor Subl.  Digitor(III  &  IV 

Flexor  Subl.  Digit.  (Inde\ 
and  Fifth  Finger) ' 

Ulnar  Nerv 

Palmaris  Bre 

Abductor  Digiti  Minimi 

Flexor  Minimi  Digiti 

Opponens  Minimi  Digit 


Musculocutaneous  Nerve 

Biceps  Muscle 
Brachialis  Anticul 


Median  Nerve 
.Supinator  Longus 

Pronator  Teres 
Flexor  Carpi  Radialis 

^  Flexor  Sublimis  Digitorum 

Flexor  Pollicis  Longus 
Median  Nerve 

Abductor  Pollicis  Brevis 
Opponens  Pollicis 

Flexor  Pollicis  Brevis 

Adductor  Pollicis 


Fig.  15. — Motor  Points  on  Inner  Surface  of  Arm.     (Erb.) 


Fig.  16. — Extreme  Atrophy  of  Thenar  Muscles — "  Ape  Hand."     (Duchenne.) 


26 


THE  NERVOUS  DISEASES   OF  CHILDREN. 
Muscles  of  Arm,  Forearm,  and  Hand. — Continued. 


Name  of 
Muscle. 


Thenar 
muscles : 

Exte  n  s  o  r 
pol  1  i  c  i  s 
brevis. 


Exte  n  s  o  r 
pol  1  i  c  i  s 

longus. 


Abductor 
pol  lie  is 
longus. 


Normal 
Function. 


Extends  first 
phalanx  and 
abducts  meta- 
carpal b  one  ; 
acts  with  ad- 
ductor polli- 
cis  longus. 

Extends  both 
phalanges  of 
thumb  ;  al  so; 
adduction  of 
meta  c  a r  pall 
bone  and 
b  ackw  a  r  d 
movement  of 
thumb. 

Abduction  of 
metacar  p  al 
bone  ;  aids  in 
flexion  of 
hand. 


Symptoms  of  Defi- 
cient action. 


Innervated 
by 


Impairment  of  ex-  M  usculo- 
tension   and   ad-    spiral, 
duction ;     flatten- 
in  g    of   ball   of 
thumb. 


Deficient  extension 
and  ad  d  uc  t ion ; 
second  phalanx  is 
flex  e  d  toward 
first. 


Deficient  abduc- 
tion of  meta- 
carpal bo  n  e  ;  if 
this  muscl  e  and 
extenso  r  pollicis 
br  e  v  i  s  are  par- 
alyzed adduction 
results. 


Musculo 

spiral. 


Opposition  No     opposition  Median, 
of  thumb.      '■  movement. 


M  usculo- 
spiral. 


Represented 


First  dorsal. 


Diseases  in 
which   Muscle  is 
commonly  In- 
volved. 


As  b  e  f  o  r  e  ; 
more  especi- 
ally in  amyo- 
trophies and 
neuritis. 


First  dorsal.  As  above. 


Musculo-  First  dorsal, 
spiral. 


Abduct  o  r  "J 

poll  ic  i  s 

brevis. 
Opponens 

pol  1  i  c  i  s 

and  outer 

portion  of 

the  flexor; 

brevis. 
Abduct  or, Flex  first  pha- N  o    flexion;    if  Median  and 

pollicis;  lanx  and    ex-,  muscles  are    par-    ulnar. 

br  e  v  i  s  ; 

flexor 

brevis 

and  ad- 
ductor. 


Flexor 
poll i  c  is 
longus. 


lanx  and    ex-    muscles  are    par 
tend   seco  n  d    alyzed   and    atro- 
phalanx   (like    phied,  ape  hand  is 
interossei,    formed.  (Fig.  16. ) 
also  have   an 
abduction 
and    adduc- 
tion action. 
Fie  x  e  s    end 
phalanx. 


No  flexion   of  end 
phalanx. 


Median. 


First  dorsal. 


As  above. 


As  above. 


As  above. 


As  above. 


INTRODUCTION— METHODS   OF  EXAMINATION.  27 


Fig.  17. — Extreme  "  Main  en  Griffe,"  after  Injury  to  Ulnar  Nerve  A.     Subluxation  of 
Hypertrophied  Heads  of  Metacarpal  Bones  B.     (Duchenne.) 


Fig.  18. — Slight  Atrophy  of  Interossei,  and  Beginning  of  "Clawed  Hand." 
(Duchenne.) 


28 


THE  NERVOUS  DISEASES   OF  CHILDREN. 


Muscles  of  the  Pelvic  Girdle  and  Lower  Extremities. 


1          _.. 

Diseases  in 

Name  of 

Normal 

Symptoms  of  Defi- 

Innervated 

Represented     which  Muscle  is 

Muscle. 

Function. 

cient  Action. 

by 

in                commonly  In- 
volved. 

Glute  al 

Gluteus   maxi- 

Inferior  Glu- 

Fi r  s  t    a  n  d  In  progressive 

muscles. 

mus  =  exten- 

teal  (sacral 

second    sa- 

atr  o  p  h  l  e  s  ; 

sion  of  leg  at 

plexus). 

c  r  a  1     seg- 

these muscles 

hip  and  slight 

ments. 

are  hypertro- 

rotation    out- 

phied or  atro- 

ward. 

No     extension     of 
thigh ;    great    dif- 
ficulty   in    climb- 
ing; no  abduction 
of  thigh;  waddling 

phied  in  vari- 
ous forms    of 
dystr  o  phy ; 
mye  litis   of 
lower  cord; 
poliomyelitis ; 
traumatic    in- 
juries to  cord 

Gluteus    med- 

gait,  exaggerated  Gluteal     su- 

and  cauda 

ius    et    mini- 

movement of  pel- 

perior. 

equina. 

mus  =  abduc- 

vis. 

tion    of    leg  ; 

if    leg  is    ex- 

tended also 

lateral  move- 

ment of  trunk. 

External 

Outward  rota- 

Deficient   outward 

1 

Fifth    lum- 

As above. 

rotators  : 

tion  of  thigh. 

rotation;    leg 

bar. 

Pyrifo  r- 

turned  inward. 

Sacral 

mis. 
Gemelli. 

plexus 
(muscular 

Quadratus 

branches). 

femoris. 

Internal 

obturator. 

J 

Extern  al 

Obturator 

obturator. 

nerve  (lum- 

bar plexus) 

Ilio-psoas. 

F 1  e  x  i  on  and 

~|  Flexion  diffi- 

Crural  (lum-  Fourth  lum- 

outward rota- 

1   cult;     in  bed 

bar  plexus)    bar. 

tion  of  thigh. 

1    thigh  cannot  be 
\  flexed;    diffi- 

Tensor 

Flexion  and  in- 

culty    rising 

Superior 

As  above. 

fasciae  la- 

ward  rotation. 

from  horizontal 

gluteal. 

tae. 

J    position. 

Adduc  tor 

Adduction     of 

No   adduction; 

Obtu  r  a  t  o  r  Third     lum- 

muscles. 

thigh. 

thigh  rolls  out- 
ward. 

nerve,  great 
sciatic    and 
crural. 

bar. 

Sartorius. 

Flexion  of  hip 

Flexion  impaired  ; 

Crural. 

Third     lum- 

As above. 

and  knee, 

acts  imperfectly. 

bar. 

and    slight 

outward  rota- 

tion of  thigh. 

Quadri- 

Extensor of  leg 

Leg  cannot  be  ex- 

Crural. 

Fourth  lum- 

As above ; very 

ceps  fe- 

(rectus femor- 

tended ;  to  test  it 

bar. 

fre q u e n t  in 

moris. 

is   also  flexes 
hip). 

ask  patient,  who  is 
lying    down   with 
hip  bent,  to  stretch 
out  the  leg  ;  when 
patient   is    sitting 
down     to    extend 
leg  ;  or  try  to  flex 
leg   while  patient 
extends  forcibly  in 

poliomyelitis. 

the  sitting  posture 

with   leg  hanging 

down. 

INTRODUCTION—  METHODS   OF  EXAMINATION.  2<j 


Sciatic  Nerve 
kBiceps  Femoris  (Long  Head 
Biceps  Femoris  (Short  Head 

Peroneal  Nerve 


Gastrocnemius  (Outer  Head; 


Flexor  Hallucis  Longus 


Gluteus  Maximus 


Adductor  Magnus 

Semitendinosus 

Semimembranosus 


Tibial  Nerve 

Gastrocnemius  (Inner  Head) 
Soleus 

Flexor  Digit.  Comm.  Longuc 
Tibial  Nerve 


Fig.  19. 


Crural  Nerve 

Obturate  r  Nerve 

Pectinus 

Adductor  Magnus 

Adductor  Long. 

Crural  Nerve 


Tensor  Fasciae  lata? 


i Internif 


Fig.  20. 


30  THE  NERVOUS  DISEASES   OF  CHILDREN. 

Muscles  of  the  Pelvic  Girdle  and  Lower  Extremities. — Continued. 


Name  of 
Muscle. 


Normal 
Function. 


Biceps; 
Semite  n- 
dinos  u  s 
and  semi- 
membra- 
nosus. 


Gastrocne- 
mius (al- 
so plan- 
tariusand 
soleus). 


Peron  e  u  s 
longus. 


Anterior 
tibial 
mus  c  1  e  s 
(tib  ialis 
ant  i  c  u.  s , 
exten  s  o  r 
d  igi  t  o- 
rum,  and 
exten  s  o  r 
pollicis 
longus). 


Poster i  or 
t  ib  i  al 
muscle. 


Peron  e  u  s 

brevis. 
Interossei 
pedis     et 
lumbri- 
cales. 


Adductor ; 
flexor 
brevis 
and  ab- 
ductor 
hallucis. 


Symptoms  of  Defi- 
cient Action. 


Flexors  of  leg, 
and  extensors 
of  hip  in  ordi- 
nary walking, 
not  in  climb- 
ing stairs. 


Flexion  and 
adduction  of 
foot  ;  toes 
point  inward; 
first  phalang- 
es are  extend- 
ed; last  pha- 
1  a  n  g  e  s  in 
plantar  flex- 
ion. 

Slight  flexion, 
chiefly  abduc- 
tion of  foot  ; 
elevates  outer 
margin  of 
foot. 

Extensors  o  f 
foot  and  toes; 
tibialis  anti- 
cus  is  also  ad- 
d  u  c  t  o  r ;  ex- 
tensor digito- 
r  u  m  in  ad-j 
dition  to  mov- 
ing toes,  also1 
elevates  outer 
ridge  of  foot 
and  abducts 
foot. 

Adduction  o  f 
f oo  t  ;  outer 
margin  of 
foot  becomes 
convex. 

Abduction  o  f 
foot. 

Abduction  and 
adduction  of 
toes ;  flexors 
of  first  pha- 
lanx ;  extend 
second  and 
third  phalanx. 


Deficient  flexion  ; 
action  of  quadri- 
ceps may  cause 
excessive  ext en- 
sion;  in  standing ; 
thigh  is  flexed  to 
excess  ;  trunk! 
moved  backward. ' 

Deficient  flexion  of 
foot ;  heel  cannot 
be  raised  ;  cannot 
stand  on  tiptoes, 


Deficient  abduc- 
tion ;  plantar  arch 
lessened;  in- 
creased by  con- 
tracture. (Figs. 
21-24)  ;  flat-foot ; 
walking  tiresome. 

Deficient  exten- 
sion; "drop-foot," 
toes  scrape  floor  ; 
to  clear  this,  ex- 
cessive flexion  at 
knee  and  hip; 
contr  a  c  t  u  r  e  of 
flexors  and  pes 
equinus  or  equi- 
novarus.  (Figs. 
ii,  26,  27.) 


Innervated 
by 


Sciatic. 


Deficient  abduc- 
tion or  adduc- 
tion; deformities 
result  from  de- 
ficiencies. 


Internal  pop- 
liteal. 


Peroneal. 


Anterior  tib- 
ial. 


Represented 


Fifth 
bar. 


Fifth 
bar. 


lum- 


Diseases  in 
which  Muscle  is 
commonly  In- 
volved. 


As  before. 


lum-  Most  promi- 
nently affect- 
ed in  dystro- 
phies and  in 
poliomyelitis. 


Flex  first  pha- 
lanx of  big  toe 
and  extend 
second. 


J 

Abduction  and  ad- 
duction of  t  o  e  s 
deficient  ;  paral- 
ysis of  interossei ; 
hyperextension  of 
first  phalanges ; 
second  and  third 
flexed  (clawed 
foot).     (Fig.  25.) 

Deficient  flexion  of 
toes  ;  foot  cannot 
be  pushed  off 
ground  easily. 


Pos  t  e  rior 
tibial  nerve 


Peroneal. 


Posterior 
tibial. 


First  and 
second  sa- 
cral. 


Fifth  lum 
bar,  first  sa- 
cral. 


Posterior 
tibial. 


First  and 
second  sa- 
cral. 


First  and 
second  sa- 
cral. 


As  above,  but 
particularlyin 
poliomyelitis; 
per  i  p  h  e  r  a  1 
neuritis ;  fre- 
quent also  in 
peroneal  form 
of  progressive 
muscular 
atrophy. 


-  As  above. 


INTRODUCTION— METHODS   OF  EXAMINATION. 


31 


FlG.  21. — Submetatarsal  Prominence.  A 
Lower  than  Normal,  and  Increased 
Plantar  Arch,  resulting  from  Contract- 
ure of  Peroneus  Longus. 


Fig.  22. — Exhibiting,  in  Addition,  Promi- 
nence of  the  Tendon  of  the  Peroneus 
Longus  F.     (Duchenne.) 


Fig.  23. — Plantar  Surface    Fig.  24. — Same   Foot.     Fig.  25. — "Clawed  Foot"    from 


of  Same  Foot,  exhibit- 
ing Changes  due  to  Con- 
tracture of  Peroneus 
Longus,  shortening  of 
Transverse  Diameter  A 
C,  and  Torsion  of  Foot. 


Valgus  Position  due  to 
Same  Cause. 


Atrophy  of  Interossei  and  Other 
Intrinsic  Muscles  of  Foot.  (Du- 
chenne.) 


32 


THE  NERVOUS  DISEASES   OF  CHILDREN. 


Fig.  26. —  Pes  Equinus  in  a  Boy 
Five  Years  of  Age  from  Atrophy 
of  Tibialis  Anticus. 


Fig.   27.  —  Pes    Equinus    of 
Eight  Years  Duration. 


Tibialis  Amicus 
Extensor  Digit.  Commun.  Longus. 


Peroneus  Brevis  g> 


Extensor  Hallucis  Longus 


Dorsal  Interossei  { 


Peroneal  Nerve 


Gastrocnemius  (Outer  Head). 
Peroneus  Longus 


Flexor  Halluci*  Longus 


Extensor  Digit  Comm.  Brev. 
Abductor  Minimi  Digiti 


Fig.  28.— Motor  Points.     (Erb.) 


INTRODUCTION— METHODS   OF  EXAMINATION. 
Muscles  of  Back  and  Trunk. 


33 


Name  of 

Normal 

Symptoms  of 

Innervated 

Represented 

which  Muscle  is 

Muscle. 

Function. 

Deficient  Action. 

by 

in 

commonly  In- 
volved. 

Erector 

Extens  ion   of 

Lordosis   of  lower 

Dorsal 

Second  to 

In    spinal    dis- 

spinse: 

lower     dorsal 

spine;  perpendic- 

nerves. 

12th  dorsal 

eases    and 

sacro- 

and     lumbar 

ular    line     from 

segments. 

progr  essi ve 

lumbal- 

vertebras. 

shoulder  falls  be- 

muscular   at- 

is ;    lon- 

ll  i  n  d  os  sacrum; 

rophies. 

gissimus 

unilateral  palsy 

dorsi. 

causes    deflection 
of   spine    toward 
sound  side.    (Fig. 

3°) 

Lateral  movements 

Quadratus 

Deflects  lower 

Lumbar 

As  above. 

1  u  m  bo- 

p  o  r  tio  n    of 

of  lower  vertebras 

nerves. 

rum. 

spine   lateral- 
ly. 
Aid  in  expira- 

imperfect. 

Abdomin- 

Lordosis, with  pro- 

Dorsal 

S  ec  on  d  to 

As  above. 

al   mus- 

tion ;    also  in 

trusion    of    nates 

nerves. 

12th   dorsal 

cles. 

voiding  blad- 
der and  strain- 
ing at    stool : 
in  k  e  e  p  i  n  g 
vertebrae     i  n 
position. 

and    a  b  d  om  e  n  ; 
other  actions  defi- 
cient ;     cannot 
straighten  up 
fr  0  m  recumbent 
position     without 
assistance  of 
hands. 

segments. 

Serratus  Magnus 


Obliquus  Abdominis 
Externus 


Transveraus  Abdom 
inis 


-    Rect.  Abdominis 


Fig.  29.— Motor  Points  of  Trunk,     (Bernhardt.) 


34 


THE  NERVOUS  DISEASES   OF  CHILDREN. 


Paralysis  of  individual  muscles  is  not  as  frequent  in  the  lower  extremities 
as  in  the  upper ;  large  groups  of  muscles  (the  anterior  tibial,  the  posterior 
tibial)  are  often  paralyzed  together.  Symmetrical  palsies  are  common  (in- 
volving thigh  groups,  both  post-tibial  groups,  etc.).     Complete  paraplegia  of 

the  lower  extremities  is  seen  in 
some  spinal  and  cerebral  lesions  of 
children  (spastic  birth  palsies,  dor- 
sal myelitis  (poliomyelitis  and  my- 
elitis), and  in  multiple  neuritis.  Pa- 
ralysis may  be  flaccid  (spinal  or 
peripheral)  or  spastic  (cerebral,  or 
spinal).  If  spinal  and  spastic,  lesion 
is  in  lateral  column. 

To  test  the  exact  extent  of  pa- 
ralysis, the  child  is  to  be  placed  on 
its  back ;  if  it  does  not  move  the 
limbs  at  all  voluntarily,  suspicion 
of  very  great  paralysis  or  of  exces- 
sive pain  is  a  safe  one. 

If  there  is  total  paralysis,  child 
cannot  hold  the  leg,  even  if  it  be 
raised  a  few  inches  only ;  deter- 
mine whether  child  can  abduct,  ad- 
duct,  flex,  or  extend  the  various 
parts.  Failure  to  abduct  may  be 
due  to  contracture  of  adductors ; 
so  also  incomplete  extension  of 
knee  may  be  due  to  contracture  of 
flexors  of  knee.  Contractured  mus- 
cles must  be  considered  with  par- 
ticular care.  In  very  young  chil- 
dren, if  it  is  desirable  to  determine 
whether  there  is  any  actual  paraly- 
sis, pinch  the  toe  steadily  until  the 
child  draws  the  leg  away,  or  may 
be  expected  to  do  so. 

With  older  children  special  tests 
can  be  made.  Ask  child  to  keep 
thigh  flexed  while  you  oppose ;  to 
test  flexors  of  thigh,  rest  your  hands 
on  its  knee  and  ask  it  to  push  it  away  by  lifting  up  thigh  ;  to  test  extension  of 
thigh  ask  it  to  climb  on  a  chair  or  observe  whether  it  raises  hip  in  walking. 
Place  the  child  on  a  table  and  ask  it  to  swing  leg  forward  and  backward ; 
to  keep  leg  straight  while  the  physician  endeavors  to  flex  it  (for  extensors  of 
leg),  or  to  keep  leg  flexed  while  physician  attempts  to  straighten  leg  (for 
flexors  of  leg).  To  test  flexors  or  extensors  of  foot  and  toes,  it  should  be 
asked  to  perform  dorsal  and  plantar  flexion  with  and  without  resistance. 


Fig.  30. — Patient  showing  Lordosis  of  Verte- 
bral Column,  due  to  Weakness  of  the  Ex- 
tensors (Erector)  of  the  Spine. 


INTRODUCTION— METHODS   OF  EXAMINATION.  35 

To  raise  on  tiptoes,  or  to  raise  toes  while  keeping  heel  on  ground  are  good 
tests  for  the  posterior  and  anterior  tibial  groups.  Paralysis  of  certain  groups 
is  attended  by  contractures  of  others.  Deformities  of  the  feet  (pes  equinus, 
equino-varus,  and  valgus)  result  from  paralysis  and  contractures  of  the  flex- 
ors, extensors,  and  of  the  intrinsic  muscles  of  the  feet. 

Examination  of  the  gait  is  of  the  utmost  importance  ; 
an  attempt  should  be  made  to  have  the  child  walk  ;  and 
even  if  it  cannot  walk,  the  position  of  the  legs  in  stand- 
ing, or  in  attempting  to  walk,  is  often  quite  characteristic. 
Cross-legged  position  and  cross-legged  progression  occur 
in  children  (spastic  paraplegia  or  diplegia) ;  the  gait  may  be 
paretic,  spastic,  ataxic,  or  a  combined  form  of  all  three.  If 
the  gait  is  simply  paretic  the  child  drags  its  legs  carefully, 
walking  slowly  and  without  raising  the  feet.  The  spastic 
gait  is  common  in  the  hemiplegias  and  other  cerebral  palsies 
of  childhood ;  also  in  spinal  palsies  (cervico-dorsal  myelitis 
Avith  involvement  of  the  lateral  columns).  The  knee  is  stiff, 
the  leg  is  moved  as  a  whole,  and  the  child  walks  either  on 
the  ball  of  foot  or  on  its  toes.  The  ataxic  gait,  variously 
modified,  occurs  in  neuritis  in  cerebellar  disease,  and  in  hered- 
itary ataxy.  In  walking  the  child  reels,  its  legs  are  moved 
extravagantly,  and  it  walks  with  a  broad  base.  A  swinging 
gait  is  very  frequent  in  poliomyelitis.  The  gait  varies  accord- 
ing to  the  muscles  affected  ;  in  dystrophies,  the  gait  is  apt  to 
be  waddling,  or  a  mixture  of  the  waddling  and  swinging  gait. 

To  be  efficient,  muscular  action  must  be  well  directed.  The  contractions 
of  muscles  may  be  entirely  normal,  but  unless  the  functions  of  muscles  act- 
ing together  are  properly  co-ordinated,  the  contractions  may  fail  of  their  prop- 
er effect.  In  all  movements  (in  walking,  standing,  writing,  grasping,  talk- 
ing), co-ordination  of  the  muscles  therein  concerned  is  essential.  We  speak 
of  inco-ordination  or  of  ataxia,  if  movements  go  wide  of  the  mark.  If  a  per- 
son, instead  of  touching  the  end  of  the  nose  with  the  tip  of  the  fingers  (eyes 
being  closed),  fumbles  all  over  the  face,  he  has  ataxia  of  the  upper  extrem- 
ities ;  and  if  instead  of  preserving  his  balance  accurately  in  walking,  a  person 
sways  from  one  side  to  the  other,  and  to  keep  his  balance  at  all  walks  with  a 
broad  base,  that  person  has  an  ataxic  gait.  The  ataxia  may  result  not  merely 
from  inco-ordination  of  the  muscles,  but  from  insufficient  sensory  (muscular) 
impressions  of  the  position  in  space  of  various  parts  of  the  body.  If  a 
person  is  asked  to  cross  one  knee  over  the  other  (with  eyes  closed),  he  must 
know  exactly  where  the  other  knee  is,  if  he  is  to  perform  the  movement  with 
nicety  ;  and  he  must  be  able  to  gauge  the  exact  extent  of  muscular  action  to 


$6  THE   NERVOUS  DISEASES   OF   CHILDREN. 

be  employed.  With  the  assistance  of  vision  muscular  co-ordination  is  more 
perfect.  In  children  ataxia  is  less  frequent  than  in  adults  ;  it  occurs  in  mul- 
tiple neuritis,  in  hereditary  ataxy,  and  in  cerebellar  disease.  (In  the  last- 
named,  there  is  considerable  reeling  in  addition  to  mere  ataxia  ;  there  is 
inco-ordination  and  great  uncertainty.) 

Before  leaving  the  muscular  system  it  is  well  to  note  the  tonus  of  the 
muscles.  In  children  this  is  of  importance ;  for  by  the  mere  handling  of  a 
leg  the  experienced  physician  will  be  able  to  determine  whether  muscles  are 
normal,  flabby,  atrophied,  or  contractured.  Every  normal  muscle,  if  struck 
lightly  with  a  percussion  hammer,  or  with  a  finger,  will  exhibit  a  wavelike  con- 
traction. This  mechanical  excitability  may  be  increased,  so  that  the  entire 
muscle  contracts  forcibly  upon  the  slightest  tap  (tapping  the  quadriceps 
femoris  may  produce  movement  like  that  of  the  knee-jerk) ;  or  instead  of 
single  contractions,  small  fibrillary  tremors  may  be  observed  on  tapping 
atrophied  or  degenerated  muscles. 

Sensory  Disturbances  are  not  easily  determined  in 
children.  Many  of  the  finer  tests  cannot  be  applied  at  all, 
for  young  children  are  not  sufficiently  intelligent  to  give 
reliable  answers.  The  disturbances  of  sensation  may  be 
either  subjective  or  objective. 

i.  Subjective  sensations  may  vary  according  to  character 
of  sensory  perception  ;  thus  we  may  have  subjective  sensa- 
tions of  heat,  of  cold,  of  pressure  ;  if  these  sensations  are  in- 
tense, they  may  be  equivalent  to  pain  (Goldscheider) ;  there 
may  also  be  perverted  subjective  sensations,  such  as  formi- 
cations and  other  paresthesias  (feeling  as  though  a  part 
were  swollen,  enlarged,  unusually  heavy  and  the  like).  Pain 
may  vary  in  character  (boring,  cutting,  thumping,  burn- 
ing) ;  its  description,  as  Goldscheider  has  shown,  depending 
very  largely  upon  well-known  sensory  impressions  that 
have  been  associated  with  pain.  Distribution  of  pain  will 
vary  ;  it  may  follow  the  distribution  of  nerves  (neuralgia 
and  neuritis);  it  may  be  restricted  to  definite  areas,  and 
such  areas  of  pain  may  hold  definite  relations  to  disease 
in  distant  parts  (according  to  Head,  Dana,  Goldscheider, 
and  others).  Pain  persistently  referred  to  one  spot  is  desig- 
nated as  Topoalgia. 

2.  In  testing  for  objective  disturbances  of  sensations, 
we  must  note  that  ordinary  sensation  is  complex  in  char- 
acter ;  that  we  have  perception  of  touch,  of  pain,  of  heat 
and  cold,  of  the  active  and  passive  movements,  of  pressure 


INTRODUCTION— METHODS    OF  EXAMINATION.  37 

and  of  resistance.  There  may  be  complete  or  partial  loss 
of  these  various  sense  perceptions. 

The  simpler  the  tests  the  better;  the  aesthesiometer  is 
superfluous.  To  determine  tactile  sense,  passing  cotton 
over  the  skin  is  generally  quite  sufficient;  or  still  better,  let 
the  physician  close  his  eyes  and  touch  the  patient  ever  so 
lightly;  his  own  perception  of  contact  will  tell  whether 
that  of  the  patient  is  above  or  below  his  own ;  asking  the 
patient  to  state  in  which  direction  a  finger  is  being  passed 
is  another  good  test ;  a  correct  answer  implies  normal  tac- 
tile perception  at  every  point ;  writing  numbers  on  the  skin 
may  be  tried  in  older  children.  For  pain,  we  may  test  by 
pricking  with  a  pin,  by  using  the  faradic  current,  by  pinch- 
ing and  the  like.  In  all  these  tests  the  child's  facial  expres- 
sion.should  be  studied  carefully.  To  determine  the  tem- 
perature sense  it  is  best  to  employ  test-tubes  with  hot  and 
cold  water,  or  to  place  metallic  objects  on  the  skin  and  see 
whether  differences  in  heat  or  cold  can  be  made  out.  Gold- 
scheider  has  shown  that  there  are  special  areas  in  the 
skin  for  the  perception  of  pressure,  of  heat  and  of  cold ; 
and  that  the  acuteness  of  sensory  perception  varies  in  dif- 
ferent areas. 

His  test  for  minimum  or  maximum  heat  and  cold  per- 
ceptions are  not  practicable,  however,  at  the  bedside — least 
of  all  in  children. 

The  term  "muscular  sense"  led  to  misconceptions.  It 
included  a  perception  of  passive  movements,  of  active 
movements ;  of  pressure  and  of  resistance,  and  a  perception 
of  the  position  of  parts. 

To  test  the  perception  of  passive  movements  it  is  best  to  take  the  joint 
firmly  between  the  two  hands  ;  then  move  the  joint  slightly  and  ask  whether 
patient  knows  what  has  been  done  ;  a  deficiency  of  this  sense  can  be  made  out 
easily.  This  test  is  an  important  one,  for  many  cases  of  ataxia  are  depend- 
ent upon  the  loss  of  this  special  perception.  To  test  perception  of  weight,  it 
is  best  to  use  rubber  balls  of  same  size,  but  filled  with  varying  quantities  of 
shot.  The  balls  are  placed  on  the  hand,  on  the  leg,  etc.,  and  the  patient  is 
asked  to  determine  which  is  lighter  or  heavier.  The  patient's  knowledge  of 
the  position  of  parts  is  tested  by  placing  one  part  (a  leg,  a  finger),  in  a  def- 
inite position,  or  raising  it  to  a  definite  height  (eyes  closed)  and  then  asking 
patient  to  put  a  symmetrical  part  in  relatively  the  same  position. 

Loss  of  sensation  is  termed  anaesthesia  ;  diminished  sensation,  hypaesthe- 


38 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


mentantt-a 
Fig.  32.  Fig.  33. 

Distribution  of  the  Sensory  Nerves.     (Freund.)     Figs.  31-37. 


INTRODUCTION— METHODS    OF  EXAMINATION.  39 


Fig.  37. 


40  THE  NERVOUS  DISEASES   OF  CHILDREN, 

sia ;  and  increased  sensory  perception  is  termed  hyperesthesia.  Disturb- 
ances of  sensation  may  vary  with  the  distribution  of  sensory  nerves  (Figs. 
6,  31-37).  Hemianassthesia  implies  loss  of  sensation  in  one  half  of  the  body. 
This  is  rare  in  children,  and  occurs  chiefly  in  hysterical  affections.  Anoma- 
lies of  temperature  sense  occur  in  some  cord  affections  and  in  cases  of  neu- 
ritis. The  stereognostic  sense  implies  a  correct  perception  and  appreciation 
of  an  object  through  the  cutaneous  senses  (with  eyes  closed  the  patient  is  to 
recognize  familiar  objects — such  as  coins,  keys,  buttons,  placed  on  different 
parts  of  the  body).  Unless  all  sense  perceptions  and  associated  memories 
are  normal,  astereognosis  may  be  developed. 

Examination  of  Reflexes. — We  distinguish  between  the  deep  or 
tendon  reflexes,  and  the  superficial  or  cutaneous  reflexes.  The  former  con- 
stitute the  more  important  class.  Among  the  deep  reflexes  the  patella-tendon 
reflex  or  knee-jerk  has  the  greatest  clinical  significance ;  next  in  order  is  the 
ankle  clonus,  or  Achillis-tendon  reflex,  and  in  many  cases  the  behavior  of 
the  wrist  reflex,  the  triceps  reflex,  and  even  the  jaw-jerk  is  carefully  to  be 
considered.  In  children  it  is  more  difficult  than  in  adults  to  elicit  the  deep 
reflexes,  for  the  former  cannot  relax  their  muscles  easily,  and  thus  inhibit  to  a 
degree  every  reflex  movement.  In  testing  for  the  knee-jerks  and  for  other 
reflexes,  it  is  best  to  take  the  child  unawares,  before  it  has  learnt  to  know 
what  the  test  means.  While  busying  one's  self  with  other  parts  of  the  body, 
or  while  talking  to  the  child,  if  the  leg  is  in  a  favorable  position,  strike  the 
tendon  smartly,  and  the  reflex  movement  will  ensue  if  the  conditions  are 
normal.  If  this  first  test  does  not  succeed,  seat  the  child  at  the  edge  of  a 
table  with  its  legs  dangling,  and  then  quickly  strike  the  tendon.  If  the  child 
continues  to  inhibit  the  reflex,  I  am  in  the  habit  of  placing  my  left  hand  be- 
tween the  crossed  knees  of  the  child  in  such  a  way  as  lightly  to  grasp  the 
posterior  surface  of  the  upper  leg.  The  child  is  compelled  to  relax  its 
muscles,  and  I  can  easily  tell  whether  it  makes  the  slightest  effort  to  contract 
them.  If  the  tendon  is  struck,  the  excursion  of  the  leg  is  bound  to  follow, 
provided  the  reflex  is  present.  In  older  children  Jendrassik's  method 
(clinching  the  fists  while  the  test  is  made)  may  be  employed ;  but  it  is  still 
better  for  the  physician  to  ask  the  child  to  squeeze  his  fingers  the  very  in- 
stant he  strikes  the  tendon. 

To  elicit  the  ankle  clonus,  it  is  best  to  perform  sudden  dorsal  flexion  of 
the  foot,  while  keeping  the  knee  resting  on  the  other  hand  and  in  the  position 
of  moderate  flexion.  The  same  clonic  movements  can  be  elicited  by  striking 
the  Achillis  tendon  while  the  foot  is  in  moderate  dorsal  flexion.  Babinski 
has  suggested  that  the  patient  be  asked  to  rest  the  leg  on  a  chair ;  the  Achil- 
lis tendon  can  then  be  struck  with  ease. 

The  wrist  reflex  is  tested  by  striking  the  radial  side  of  the  forearm  near 
the  wrist ;  a  smart  tap  will  cause  contraction  of  the  supinator  and  biceps 
muscles.  The  triceps  reflex  is  elicited  by  striking  the  tendon  while  the  arm 
is  flexed.  The  "  jaw  jerk,"  a  reflex  contraction  of  the  masseter,  is  brought 
about  by  striking  the  middle  of  the  chin  when  the  mouth  is  slightly  open,  or 
by  tapping  a  pencil  laid  on  the  lower  teeth  or  jaw.  I  have  seen  this  distinctly 
present  in  a  case  of  multiple  sclerosis  in  a  child. 


INTRODUCTION— METHODS   OF  EXAMINATION.  4 1 

In  testing  the  wrist  or  triceps  reflexes,  place  the  joints 
in  a  position  of  moderate  flexion  so  as  to  give  the  muscles 
the  fairest  chance  for  contraction.  The  deep  reflexes  may- 
be present,  diminished  or  exaggerated.  The  knee-jerk  is 
present  under  ordinary  conditions;  the  mere  presence  of 
the  ankle  clonus  is  abnormal ;  and  the  reflexes  in  the  upper 
extremity  are  present  in  some  and  absent  in  other  normal 
individuals. 

The  deep  reflexes  are  diminished  or  absent  in  peripheral 
nerve  disease,  in  spinal-cord  affections  involving  the  pos- 
terior columns  and  the  gray  matter  of  the  cord  ;  in  disease 
of  the  cerebellum.  They  are  exaggerated  in  diseases  of  the 
brain  and  in  diseases  involving  the  lateral  columns  of  the 
cord  ;  also  in  some  functional  diseases. 

The  diminution  of  the  reflex  is  determined  easily  enough  ; 
but  the  absence  of  the  reflex  is  more  significant  than  a  mere 
diminution.  The  diminution  or  absence  of  a  reflex  may  be 
apparent  only.  Thus  the  knee-jerk  may  be  wanting  in  con- 
sequence of  contraction  of  the  posterior  flexors  of  the  leg, 
or  there  may  be  anchylosis  of  the  joint  preventing  free 
movement ;  but  in  such  cases  the  contraction  of  the  quadri- 
ceps can  be  seen  or  felt  upon  tapping  the  tendon.  In  some 
children  the  tendon  is  displaced  or  so  imbedded  in  fat  that 
it  cannot  be  struck  readily. 

It  is  more  difficult  to  state  when  a  reflex  is  exaggerated. 
In  this  matter  comparison  based  upon  experience  is  the 
best  guide.  We  can  safely  speak  of  an  exaggerated  reflex 
if  there  is  an  exceeding  quickness  of  the  response,  if  the 
slightest  tapping  produces  a  liberal  movement ;  or  if  clonic 
movement  results  from  a  single  blow  (as  in  patella  clonus). 
If  the  reflex  can  be  elicited  indirectly  by  striking  the  finger 
placed  over  the  tendon,  the  reflex  is  surely  exaggerated. 
Moreover,  if  a  child  of  three  years  or  more  is  so  seated  that 
its  feet  are  in  solid  contact  with  the  floor,  and  if  when  its 
patella  tendon  is  struck,  the  foot  is  lifted  from  the  floor, 
the  reflex  is  considerably  increased. 

Very  lively  knee-jerks  do  not  necessarily  imply  organic 
disease,  but  exaggerated  knee-jerks  plus  ankle  clonus  and 
a  Babinski  sign  are  indicative  of  this.  Any  change  of  the 
reflexes  in  one  half  of  the  body  is  always  suspicious  of 


42  THE  NERVOUS  DISEASES   OF   CHILDREN. 

organic  disease,  and  so  is  the  association  of  increased  me- 
chanical excitability  and  of  contractures  with  increase  of 
the  reflexes. 

The  superficial  or  cutaneous  reflexes  are  not  of  great  importance  in  child- 
hood. The  abdominal,  epigastric,  and  cremasteric  reflexes  are  but  poorly 
developed  in  children  ;  the  last  named  is  absent  in  many  older  children  ;  and 
no  significance  can  be  attached  to  its  presence  or  absence  unless  the  condi- 
tion is  not  the  same  in  both  halves  of  the  body.  The  plantar  reflex  (retrac- 
tion of  the  foot  on  irritation  of  the  soles)  deserves  a  few  words  of  comment. 
Like  the  other  cutaneous  reflexes  it  is  absent  in  diseases  of  the  peripheral 
nerves,  and  in  diseases  of  the  spinal  cord  involving  the  course  of  the  reflex 
arc ;  if  the  lesion  is  higher  than  the  level  through  which  the  arc  passes,  the 
reflex  may  be  exaggerated,  but  this  is  not  as  constant  a  feature  as  is  the  case 
with  the  tendon  reflexes.  In  all  unilateral  brain-lesions  the  cutaneous  re- 
flexes are  absent  as  a  rule  on  the  paralyzed  side.  The  plantar  reflex  is  gen- 
erally absent  in  a  condition  of  coma  (as  well  as  in  deep  sleep),  and  its 
presence  or  absence  may  give  a  clew  as  to  the  degree  of  coma  in  a  given  case. 

The  Babinski  phenomenon  (dorsal  flexion  of  the  big  toe  following  irrita- 
tion of  the  sole  of  the  foot)  is  an  important  symptom  pointing  to  involve- 
ment of  the  pyramidal  tract ;  it  is  well,  however,  to  bear  in  mind  that  in 
children  under  four  years  of  age  a  dorsal  flexion  of  the  toes  is  the  normal 
reflex. 

ELECTRICAL   EXAMINATION.* 

The  distribution  of  a  paralysis,  the  flaccid  or  spastic  con- 
dition of  the  muscles,  the  behavior  of  the  reflexes,  may  en- 
able us  to  make  an  accurate  diagnosis  in  many  cases,  but  in 
many  more  the  diagnosis  cannot  be  safely  established  until 
a  careful  electrical  examination  has  been  made. 

The  chief  object  of  such  an  examination  is  to  determine 
the  response  of  various  muscles  and  nerves  to  the  faradic 
and  galvanic  currents,  and  to  compare  such  response  with 
the  conditions  obtaining  during  health. 

The  younger  Westphal  has  shown  that  the  nerves  and 

*  The  author  cannot  undertake  to  give  a  detailed  account  of  medical  electricity  ;  for 
this  the  student  is  referred  to  the  treatises  of  de  Watteville,  of  Beard  and  Rockwell,  of 
Erb,  Lewandowski  or  Toby  Cohn  ;  also  to  the  special  chapters  in  the  text-books  of 
Gray,  Dana,  and  Gowers.  The  importance  of  a  thorough  knowledge  of  electricity 
should  not  be  overlooked.  Whatever  one  may  think  of  the  value  of  electricity  as  a 
therapeutic  agent,  there  can  be  no  question  of  its  great  merit  as  an  aid  to  diagnosis. 
Moebius  argues  that  the  experienced  neurologist  can  make  a  diagnosis  without  the 
assistance  of  electrical  tests,  and  that  such  tests  are  not  wholly  reliable.  But  thev  are 
reliable  in  fully  ninety-five  per  cent,  of  all  cases,  and  as  for  making  a  diagnosis  without 
the  aid  of  electricity,  that  may  be  possible.  A  clever  neurologist  can  also  diagnosticate 
a  cerebral  tumor  often  enough  without  the  ophthalmoscope,  yet  he  will  not  spurn  the 
corroborative  evidence  which  an  examination  of  the  papillae  may  furnish. 


INTRODUCTION — METHODS   OF  EXAMINATION.  43 

muscles  of  the  new-born,  and  of  children  up  to  the  age  of 
five  weeks,  do  not  respond  except  to  very  strong  currents, 
and  even  then  the  contractions  are  slow.  But  after  that  age 
the  normal  nerve  and  muscle  will  respond  to  faradic  and 
galvanic  stimulation.  A  knowledge  of  the  position  of  the 
motor  points  is  essential.  (See  Figs.  7,  13,  14,  15,  19,  20,  28, 
29.) 

Always  begin  the  examination  with  the  faradic  current,  and  apply  the 
one  pole  to  the  nerve  or  muscle  to  be  examined,  and  place  the  other  pole  at 
a  safe  distance,  say  on  the  sternum.  Use  mild  currents  (children  grow  very 
restless  under  strong  currents),  and  determine  the  weakest  current  with  which 
a  contraction  is  to  be  obtained,  or  whether  any  contraction  can  be  obtained 
at  all,  or  not.  Place  the  arm  or  leg  in  a  position  favorable  for  contraction 
of  the  muscles  to  be  tested,  and  make  sure  that  the  contraction  which  en- 
sues is  the  one  that  should  naturally  follow.  Do  not,  for  instance,  claim  that 
the  electrical  conditions  are  normal  if  on  applying  an  electrode  to  the  ex- 
tensor digitorum  communis,  a  flexion  contraction  of  wrist  and  fingers  ensues. 
And  if  there  is  any  doubt  as  to  the  strength  of  the  current,  let  the  physician 
apply  it  to  himself ;  he  will  be  more  certain  to  be  merciful  if  he  does ;  or  if 
there  is  a  doubt  whether  a  nerve  or  muscle  should  respond  to  a  given  strength 
of  current,  let  that  same  current  be  applied  to  the  same  point  in  the  opposite 
half  of  the  body.  Thus  we  can  compare  two  peronei  with  each  other  ;  or  we 
can  compare  a  peroneal  nerve  with  a  facial  or  a  median  nerve  of  the  same 
or  opposite  side  of  the  body,*  or  we  can  compare  the  degree  of  excitability 
with  the  figures  given  in  tabular  form  by  Stintzing  for  the  minimum  and 
maximum  currents  required  to  produce  contractions  of  various  nerves  and 

muscles.t 

A  simple  diminution  of  faradic  and  galvanic  response  of  nerves  and  mus- 
cle occurs  in  light  cases  of  peripheral  neuritis,  in  hysterical  conditions,  in 
atrophies  following  joint  disease  or  from  disuse,  and  in  some  primary  dys- 
trophies. A  simple  and  continued  increase  of  response  is  observed  practi- 
cally only  in  cases  of  tetany.  But  in  addition  to  mere  quantitative  changes 
there  are  also  changes  in  the  character  and  quality  of  the  response  to  be  ob- 
served in  cases  of  degenerative  disease  of  the  peripheral  nerves,  of  the  mus- 
cles, and  of  parts  of  the  spinal  cord.  The  changes  implying  degenerative 
disease  are  referred  to  as  the  reaction  of  degeneration. 

*  In  children  the  motor  points  may  be  obscured  by  deposits  of  fat. 

t  American  faradic  batteries  are  now  provided  with  a  sliding  scale  which  enables  the 
physician  to  record  how  far  apart  the  primary  and  secondary  coils  were  when  first  con- 
traction was  obtained.  In  recording-  the  effect  of  the  galvanic  current,  the  strength 
should  be  stated  in  milliamperes. 


44 


THE   NERVOUS  DISEASES   OF   CHILDREN. 


The  following  table  will  give  the  salient  features  of  nor- 
mal electrical  conditions  and  of  the  reaction  of  degenera- 
tion : 


Normal  Electrical  Conditions. 

Reaction  of  Degeneration. 

Nerve  and  Muscle. 

Nerve. 

Muscle. 

Faradic  cur- 
rent. 

Galvanic 
current. 

Order    of 
c  o  n  t  Tac- 
tions. 

Contractions  good  ; 
prompt. 

Contractions  prompt  and 
quick. 

i.  k.  c.  a* 

2.  A.  C.  C.  |  interchange- 

3.  A.  O.  C.  \     able. 

4.  K.  O.  C. 

5.  K.  C.  Te.  (rare). 

No  response 
(except  in 
partial   R. 
D.). 

No  response. 

No  response  (except  in  partial 
R.  D.). 

Increased  excitablity  at  first, 
then    diminished  ;    contrac- 
tions sluggish. 

With  stronger  currents. 

2:k.&q[a-c-c->k-c-c- 

3.  A.  O.  C. 

4.  K.  O.  C. 

or 
A.  C.  C.  =  K.  C.  C. 

*  K.  C.  C.  =  Kathodal  closure  contraction  ;  A.  C.  C.  =  Anodal  closure  contrac- 
tion ;  A.  O.  C.  =  anodal  opening  contraction  ;  K.  O.  C.  =  Kathodal  opening  con- 
traction ;  K.  C„  Te.  =  Kathodal  closure  Tetanus. 

The  preceding  table  states  that  in  the  reaction  of  degeneration  (R.  D.) 
the  nerves  and  muscles  fail  to  respond  to  the  faradic  current,  but  that  the 
muscles  continue  to  respond  to  the  galvanic  current  in  altered  fashion  ;  first 
of  all,  the  contractions  are  sluggish,  and  this  is  the  most  important  point,  and 
secondly,  the  order  of  contractions  is  reversed  ;  the  anodal  closure  contrac- 
tion can  be  obtained  with  weaker  currents  than  are  required  for  the  kathodal 
closure  contraction. 

The  mistake  is  commonly  made  to  suppose  that  there  is 
no  reaction  of  degeneration  present  unless  the  galvanic  order 
of  contractions  is  reversed  ;  this  occurs  in  the  majority  of 
typical  cases,  to  be  sure ;  but  the  failure  of  response  to  the 
faradic  current  is  early  evidence  of  a  reaction  of  degener- 
ation, and  often  precedes  the  development  of  abnormal  gal- 
vanic conditions. 

In  first  examinations  the  faradic  test  is  therefore  much 
the  more  important. 

Many  recent  authors  (including  Remak)  agree  further- 
more in  considering  the  sluggishness  of  contraction  much 
more  significant  of  degenerative  changes  than  the  reversal 
of  the  normal  formula. 


INTRODUCTION — METHODS   OF  EXAMINATION. 


45 


It  should  be  remembered  also  that  muscles  may  be  so 
much  atrophied  as  to  give  no  contraction  to  the  strongest 
galvanic  current. 

A  partial  reaction  of  degeneration  is  often  overlooked  ;  it  implies  a  mere 
diminution  of  faradic  excitability  of  nerve  and  muscles,  but  the  response  of 
the  muscle  on  direct  galvanic  excitation  is  slow,  and  the  formula  may  be  re- 
versed. Even  if  the  faradic  response  is  present,  it  is  important  to  make  sure 
that  the  galvanic  response  is  prompt,  not  sluggish. 

The  greatest  service  which  electricity  affords  us  in  diag- 
nosis is  in  differentiating  between  cerebral  diseases  on  the 
one  hand  and  certain  spinal  and  peripheral  diseases  on  the 
other.  The  ganglion  cell  of  the  anterior  horn  and  its  ana- 
logue in  the  cranial  nerve  nuclei  are  responsible  for  the  nor- 
mal electrical  condition  of  peripheral  nerves  and  muscles. 
Lesions  involving  this  cell,  or  interfering  with  the  transmis- 
sion of  impulses  from  it  to  the  periphery  are  attended  by 
changes  in  electrical  reactions;  in  all  other  diseases  the 
electrical  conditions  are  practically  unaltered. 

Whence  it  follows  that  the  electrical  conditions  are  : 


Normal  in 

Distinctly  Altered  (R.  D.)  in 

i. 

All  cerebral  diseases,*  excepting  those 

i. 

Bulbar  paralysis  (acute  and  chronic). 

of  cranial  nerve  nuclei. 

2. 

Polioencephalitis  superior  (Wernicke). 

2. 

Diseases  of    lateral  and    posterior  col- 

3- 

Poliomyelitis  (acute  and  chronic). 

umns  of  spinal  cord. 

4- 

Progressive  amyotrophies. 

3- 

Functional  troubles. 

5- 

Amyotrophic  lateral  sclerosis. 

4- 

Mild  peripheral  troubles. 

6. 

Myelitis,   but  only  in    muscles  repre- 

5- 

In  some  forms  of  muscular  dystrophy. 

sented  in  diseased  level. 

7- 

Gliosis  and  tumor  involving  gray  mat- 
ter. 

Anterior  root  disease  (syphilis,  tumor, 
etc.) 

Vertebral   disease  (Pott's   disease,   tu- 

8. 

9- 

10. 

mor). 

Peripheral    neuritis    (traumatic,    rheu- 
matic, toxic). 

ii. 

In  some  forms  of  muscular  dystrophy. 

*  A  very  few  exceptions  have  been  reported. 

To  complete  the  examination,  be  sure  to  look  for 
trophic  symptoms  which  occur  in  many  functional  disor- 
ders as  well  as  in  disease  of  the  peripheral  nerves  and  in 
some  diseases  of  the  spinal  cord  (gliosis,  tumor) ;  and  finally 
the  conditions  of  the  vesical  and  rectal  reflexes  should  be 


46 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


recorded ;  though  in  regard  to  them  the  influence  or  lack 
of  previous  training  of  the  child  must  be  taken  into  ac- 
count. A  sudden  loss  of  control  is  often  associated  with 
serious  cerebral  and  spinal  disease. 

Lumbar  Puncture  is  a  recent  addition  to  our  methods 
of  examination.  It  is  practised  for  the  purpose  of  with- 
drawing some  of  the  cerebro-spinal  fluid.  The  examination 
of  this  fluid  may  render  valuable  aid  in  the  diagnosis  of 
cerebral  and  cerebro-spinal  affections.  Its  value  as  a  ther- 
apeutic measure  is  still  in  doubt. 

Lumbar  puncture  was  introduced  by^  Quincke.  His 
directions  are  as  follows:  The  patient  is  to  lie  on  his  left 
side,  near  the  edge  of  the  bed,  with  knees  well  drawn  up. 
The  trocar  is  to  be  inserted  in  the  median  line  between  the 
third  and  fourth  or  between  the  fourth  and  fifth  lumbar 
vertebras  (Fig.  38).     After  reaching  the  dural  sac  the  trocar 


Fig.  38.     (After  Quincke.) 
Slightly  Modified  ;  the  Horizontal  Lines  Correspond  to  the  Lumbar  Vertebra \ 


needle  is  withdrawn  and  the  cerebro-spinal  fluid  will  flow 
out.  Quincke  advises  connecting  the  canula  with  a  small 
rubber  tube  and  a  glass  manometer.  If  there  is  no  ob- 
ject in  measuring  the  pressure  at  the  time  of  tapping, 
the  trocar  is  sufficient  and  the  fluid  may  be  received  in  a 
test-tube. 


INTRODUCTION— METHODS    OF    EXAMINATION, 


47 


To  determine  the  site  for  the  puncture,  draw  a  line  at  a 
tangent  to  the  crest  of  the  ilium  ;  this  will  strike  the  fourth 
lumbar  space. 

General  or  local  anaesthesia  is  unnecessary  except  in  un- 
ruly patients.  Do  not  withdraw  more  than  20  to  30  c.  c.  at 
a  sitting.     Use  sterilized  test-tubes. 


Fig.  39.     (After  Quincke.) 


The  procedure  is  a  harmless  one  except  in  cases  of  cere- 
bellar  tumor  and  in  neoplasms  in  the  vicinity  of  the  fourth 
ventricle. 


PART   I. 
GENERAL   NERVOUS    DISEASES. 


CHAPTER   II. 

CONVULSIONS— ECLAMPSIA   INFANTUM. 

The  peculiar  seizures  so  common  in  infancy,  and  desig- 
nated as  convulsions,  constitute  a  symptom,  not  a  form,  of 
disease.  The  unusual  frequency  of  convulsions  in  early 
childhood  points  to  the  greater  excitability  in  the  child  of 
the  motor  mechanism  of  the  brain.  The  motor  centres  in 
the  cortex  are  more  apt  to  "  discharge,"  and  the  inhibitory 
power  of  the  brain  is  less  developed  than  in  the  adult. 
Eclampsia  has  been  found  to  be  a  convenient  term  for  the 
condition  in  which  convulsions  are  apt  to  occur,  but  it 
shoald  be  remembered  that  it  is  neither  a  disease  nor  a 
sufficient  diagnosis  in  any  given  case,  for  eclampsia  in 
children,  as  well  as  in  adults,  may  be  the  result  of  many 
widely  different  disorders. 

However  frequent  these  convulsive  seizures  may  be  in 
early  life,  healthy  children  are  as  exempt  from  them  as  are 
healthy  adults.  If  they  are  not  the  result  of  organic  disease 
of  the  brain,  their  occurrence  indicates  functional  involve- 
ment of  the  brain  in  connection  with  disease  in  other  parts 
of  the  body. 

A  convulsion  is  a  motor  discharge  resulting  in  muscular 
contractions  of  one  or  more  parts  of  the  body.  In  the  major- 
ity of  cases  these  convulsive  movements  are  associated  with 
a  number  of  other  symptoms ;  viz.,  an  initial  cry,  turning 
of  the  eyes  upward  or  inward,  very  sudden  loss  of  conscious- 
ness, involuntary  passage  of  urine  and  of  faeces,  prolonged 
drowsiness,  and  a  condition  of  stupor  or  of  coma.  The 
muscular  movements  may  be  tonic  at  first,  but  are  apt  to 
lead  to  clonic  spasms.  No  two  convulsive  seizures  are 
exactly  alike,  but  the  student  who  has  seen  a  single  seizure 
will  not  forget  the  main  features  of  the  condition.     The 


52  THE  NERVOUS  DISEASES   OF   CHILDREN. 

convulsion  may  come  on  without  warning,  or  the  child  may 
have  complained  of  uncomfortable  sensations  in  the  head  or 
stomach,  of  a  little  dizziness  or  of  nausea ;  the  child  gives  a 
shrill  cry  and  at  once  is  thrown  into  tonic  and  clonic  con- 
vulsions. These  may  be  partial  or  general,  unilateral  or 
bilateral.  The  legs  are  stiff,  the  arms  bend  at  the  elbow, 
the  wrists  are  flexed,  and  the  hands  clenched,  with  the 
fingers  firmly  closed  upon  the  thumb.  The  head  is  thrown 
back  or  rotated  to  one  side ;  the  back  may  be  arched,  the 
face  is  pale  at  first,  but  soon  reddens  and  the  eyes  remain 
wide  open.  The  pupils  do  not  react.  In  older  children 
the  tongue  may  be  caught  between  the  teeth,  and  bloody 


Fig.  40. — Child  of  One  Year  Photographed  During  a  Severe  Convulsive  Seizure. 

froth  may  be  noticeable  at  the  lips.  In  very  young  chil- 
dren we  have  the  ordinary  foam  at  the  mouth.  The  respi- 
ratory muscles  are  in  a  condition  of  spasm  ;  the  child  often 
turns  blue  rapidly  and  is  in  danger  of  asphyxia.  Fortu- 
nately the  tonic  spasm  soon  ceases,  slighter  clonic  convul- 
sions then  set  in,  and  after  a  little  the  child  grows  quiet, 
and  is  certain  to  lie  in  a  dazed  or  stuporous  condition  for  a 
period  that  may  vary  from  several  minutes  to  as  many 
hours  or  days.  While  the  clonic  spasm  lasts  there  is  im- 
minent danger  of  injury -to  the  child's  head  and  limbs  in 
consequence  of  the  severe  jactations,  but  this  danger  is  not 
as  serious  as  that  from  asphyxia  during  the  period  of  tonic 
spasm. 

Causes. — Convulsions  always  denote  "  cerebral  (cortical) 
irritation."     It  might  be  sufficient  to  state  that  any  morbid 


C  ONVULSIONS—ECLA  MP  SI  A    JXFA  NTUM. 

process  which  brings  about  cortical  irritation,  directly  or 
indirectly,  is  liable  to  cause  convulsions.  But  it  will  be 
better  to  enumerate  the  chief  conditions  under  which  in- 
fantile convulsions  occur. 

I.  Convulsions  occurring  within  the  first  few  days  oi 
life  are,  as  a  rule,  the  result  of  meningeal  hemorrhage,  due 
either  to  protracted  labor,  or  to  instrumental  delivery.  If 
the  child  survives,  the  injury  done  to  the  brain  often  leads 
to  the  development  of  spastic  palsies.  (Cf.  chapter  on  Cer- 
ebral Palsies.) 

II.  The  convulsions  may  be  due  to  organic  disease  of  the 
brain,  such  as  tumor,  abscess,  meningitis,  or  vascular  lesions ; 
in  all  of  these  cases  they  may  be  the  earliest  symptom  of 
the  disease,  but  other  symptoms  associated  with  them,  such 
as  headache,  paralysis,  optic  neuritis,  and  the  like,  will  be 
forthcoming. 

III.  They  may  mark  the  onset  of  any  acute  infectious 
disease.  The  initial  convulsion  in  children  may  have  the  same 
value  as  the  initial  rigor  in  the  adult.  The  former,  with  or 
without  fever,  is  the  clinical  equivalent  of  the  latter.  It  is 
common  at  the  onset  of  pneumonia,  scarlet  fever,  and  measles ; 
and  I  have  also  witnessed  convulsions  in  malarial  fevers,  and 
at  the  onset  of  chicken-pox.  The  general  practitioner  is 
very  apt  to  err  in  the  interpretation  of  such  convulsions. 
A  child  that  has  had  a  convulsion,  preceded  or  followed  by 
a  rise  of  temperature,  is  often  suspected  to  be  in  the  first 
stages  of  a  meningitis  or  of  some  other  cerebral  disease. 
While  this  is  true  in  some  instances,  the  fact  is  entirely 
overlooked  that  the  seizure  may  be  the  initial  symptom  not 
only  of  any  of  the  ordinary  infectious  diseases,  but  of  the 
acute  spinal  diseases  as  well  —  poliomyelitis,  for  instance 
(probably  an  infectious  disease). 

IV.  The  convulsions  may  be  of  reflex  origin.  Almost 
every  possible  peripheral  disturbance  has  been  supposed  by 
one  author  or  the  other  to  be  a  sufficient  cause  of  convul- 
sions. I  need  merely  mention  ocular  insufficiencies,  nasal 
obstruction,  narrow  prepuces,  not  to  call  up  a  dozen  or 
more  about  which  there  has  been  much,  and  often  bitter, 
discussion.  Of  the  influence  of  two  conditions  there  can  be 
no  reasonable  doubt.     The  first  of  these  is  dentition ;  the 


54  THE  NERVOUS  DISEASES   OF   CHILDREN. 

second,  gastro-intestinal  irritation.  There  has  been  some 
question  whether  a  tooth  about  to  cut  through  the  gum  can 
cause  an  eclamptic  seizure.  Delayed  dentition  is  so  fre- 
quently associated  with  rickets  that  the  latter  condition  is 
considered  by  many  to  be  the  chief  factor,  and  this  I  be- 
lieve to  be  true  ;  but  convulsions  do  at  times  occur  in  chil- 
dren who  present  no  tangible  signs  of  rickets.  Moreover, 
there  is  good  reason  why  a  cutting  tooth  should  produce 
convulsions  in  a  child  so  disposed,  say  by  rickets,  or  any 
form  of  exhausting  disease.  "  The  cutting  tooth  "  is  a  direct 
irritant  to  the  filaments  of  the  trigeminal  nerve,  which  car- 
ries this  irritation  easily  enough  to  the  convulsive  centres 
at  the  base  of  the  brain.  I  have  little  doubt  of  this  special 
reflex  origin  of  convulsive  seizures  since  observing  in  the 
adult  the  occurrence  of  severe  epileptic  paroxysms  in  cases 
of  trigeminal  neuralgia. 

The  influence  of  gastro-intestinal  irritation  is  well  illus- 
trated by  the  convulsions  occurring  in  the  course  of  an 
acute  or  chronic  intestinal  catarrh,  in  the  ordinary  summer 
diarrhoea  of  young  children,  or  with  the  exhausting  chronic 
diarrhoea  in  older  children.  The  same  phenomena  are  apt 
to  occur  in  the  presence  of  animal  parasites  (pin-worms, 
and,  above  all,  tape-worms),  and  I  have  known  severe  con- 
vulsions to  disappear  promptly  after  the  removal  of  a  tape- 
worm in  children  between  the  ages  of  four  and  eight  years 
never  to  return  again. 

V.  Convulsions  may  be  due  to  poisons  (organic  or  me- 
tallic) circulating  in  the  blood.  Under  this  heading  we 
may  include  the  convulsions  of  uraemia,  those  due  to  the 
presence  of  ptomaines  in  the  stomach  and  bowels,  and  con- 
vulsions produced  by  the  administration  of  lead  and  other 
metallic  poisons.  Toxasmic  convulsions  are  not  as  common 
in  the  child  as  in  the  adult,  unless,  following  recent  studies 
by  Chenbach  and  others,  we  consider  the  convulsions  of  in- 
fectious fevers  and  of  intestinal  troubles  to  be  due  to  some 
form  of  auto-intoxication. 

VI.  Convulsions  may  result  from  severe  loss  of  blood, 
from  any  exhausting  disease,  or  from  such  constitutional 
disturbances  as  scrofula  and  rickets.  A  vast  literature  has 
grown  up  on  the  subject  of  rickets  and  convulsions.    What- 


CONVULSI ONS—E  CLA  MP  SI  A    INF  A  NIL  \M. 


55 


ever  theory  one  may  be  willing  to  adopt  in  order  to  ex- 
plain this  close  relationship,  the  fact  remains  that  an  enor- 
mous percentage  of  children  having  convulsions  are  af- 
fected with  rickets. 

An  hypersemic  condition  of  the  cortex  analogous  to  the 
condition  of  the  bones  in  rickets,  is  the  only  explanation  we 
can  give  of  the  frequency  of  convulsions  during  rickets, 
unless  we  are  satisfied  to  accept  the  very  vague  statement 
that  the  latter  is  a  simple  predisposing  cause  of  convul- 
sions. 

VII.  A  convulsion  may  be  idiopathic,  hereditary,  or,  if 
you  choose,  the  first  incident  in  the  course  of  an  ordinary 
epilepsy.  This  possibility  must  be  kept  in  mind,  but  it  is  a 
more  probable  explanation  if  the  child  happens  to  be  above, 
rather  than  under,  four  or  six  years  of  age.  This  inter- 
pretation of  a  convulsive  seizure  occurring  in  a  child  should 
be  given  only  after  excluding  every  other  possible  cause. 

It  is  surprising,  but  a  fact  nevertheless,  to  which  I  have 
called  attention  a  number  of  times,  that  the  more  carefully 
we  scrutinize  cases  of  convulsions,  or  of  epilepsy  for  that 
matter,  the  fewer  of  them  appear  to  be  truly  idiopathic.  It 
will  be  well  not  to  make  the  diagnosis  of  an  incipient  epi- 
lepsy unless  we  are  informed  that  the  child  has  had  previous 
convulsions  at  somewhat  long  intervals,  or  unless  we  have 
occasion  to  observe  subsequent  convulsions  without  a  dis- 
tinct additional  cause  for  each  seizure. 

VIII.  Convulsions  may  be  due  to  traumatism.  In  some 
cases  actual  hemorrhage  over  the  motor  centres  is  the  im- 
mediate cause  of  the  convulsions.  In  other  cases  no  tan- 
gible injury  to  the  brain  has  resulted,  and  we  are  therefore 
compelled  to  regard  the  spasm  as  the  expression  of  shock. 

Theories. — Many  theories  have  been  advanced  to  ex- 
plain the  occurrence  of  convulsions.  No  theory  is  entirely 
satisfactory  ;  but  by  physiological  experiments  and  the  ex- 
periment of  disease  we  have  learned  to  know  the  conditions 
under  which  convulsions  are  most  apt  to  occur. 

There  are,  first  of  all,  the  time-honored  experiments  of 
Kussmaul  and  Tenner,  showing  that  convulsions  occur  in  a 
rabbit  after  suddenly  tying  the  cervical  arteries  and  thus 
cutting  off  the  blood-supply.     Their  conclusions  are  op- 


56  THE  NERVOUS  DISEASES   OF   CHILDREN. 

posed  to  the  older  theories  that  these  seizures  were  due  to 
an  lrypersemic  condition  of  the  brain.  The  question  arises 
whether  the  sudden  withdrawal  of  blood  deprives  parts 
of  the  brain  of  their  functions,  or  whether  it  simply  acts  as 
an  irritant  to  the  cortical  centres.  The  latter  explanation 
seems  plausible,  in  view  of  the  experiments  of  Hitzig,  Fer- 
rier,  Horsley  and  others.  A  mechanical  irritation  of  the 
pons  (floor  of  the  fourth  ventricle),  as  proved  by  Nothnagel, 
also  produces  convulsions.  It  is  evident  that  toxic  agents 
have  the  same  effect  as  the  electrical  or  mechanical  irrita- 
tion just  referred  to,  else  we  could  not  explain  the  convul- 
sions due  to  toxic  agents,  those  of  uraemia,  for  instance, 
and  those  due  to  asphyxia  from  the  accumulation  of  car- 
bonic acid  in  the  blood. 

Every  morbid  process  in  the  motor  areas  of  the  cortex, 
if  not  absolutely  destructive,  is  apt  to  cause  convulsions ; 
but  what  is  the  relation  between  the  convulsive  centres  in 
the  cortex  and  Nothnagel's  epileptic  centre  in  the  pons? 
Does  the  cortex  contain  an  actual  convulsive  centre?  If 
you  irritate  the  lower  epileptic  centre  in  an  animal  whose 
hemispheres  have  been  divided  from  the  rest  of  the  brain 
you  will  get  convulsive  spasms  of  the  entire  body.  The 
lower  centres  have  the  power,  therefore,  of  "  starting  "  con- 
vulsive seizures,  and  it  is  probable  that  they  are  under 
higher  control  in  the  fully  developed  brain,  and  so  long  as 
normal  conditions  exist.  It  is  this  power  to  control,  this 
inhibitory  force,  that  resides  in  higher  centres.  The  re- 
moval of  this  inhibition  through  disease  of  such  centres 
liberates  the  energy  of  the  lower  centres.* 

Age  and  Frequency. — By  far  the  largest  majority  of 
convulsions  occur  during  the  first  two  years  of  life.  The 
Philadelphia  Health  Reports,  as  condensed  by  Lewis,  give 
ample  proof  of  this. 

Seven  thousand  five  hundred  and  eight  deaths  among 

*  Neurologists,  the  world  over,  have  adopted  Hughlings  Jackson's  views  without  en- 
dorsing every  detail  of  his  theories.  Jackson  considers  the  central  nervous  system  to 
be  made  up  of  three  tiers  of  sensory-motor  centres.  All  parts  of  the  body  are  repre- 
sented in  each  tier,  but  the  higher  centre  controls  one  or  both  below  it,  and  has  the 
power  to  inhibit  the  discharges  of  a  lower  centre.  In  a  young  child  the  higher  tiers  do 
not  exert  a  controlling  influence,  since  they  are  imperfectly  developed  ;  hence  the  dis- 
charges are  so  much  more  frequent  than  in  later  years. 


CONVULSIONS— ECLAMPSIA    INFANTUM. 


57 


minors  (during  period  of  1876-1883  inclusive)  were  due  to 
convulsions  and  laryngismus  stridulus.     Of  these  were  : 


Under 
1  year. 

1  to  2 
years. 

2  to  s 
years. 

5  to  10 
years. 

10  to  15 
years. 

15  to  20 
years. 

Cases  of  convulsions .... 

Cases     of      laryngismus 

stridulus 

4,993 
24 

1-335 
7 

893 

9 

178 

5 

38 

26 

Total 

5.oi7 

1.342 

902 

183 

38 

26 

While  the  statistics  illustrate  a  general  truth,  some  com- 
ment is  necessary.  First,  convulsions  occurring  at  this 
early  age  are  not  necessarily  fatal,  although  those  occur- 
ring in  the  first  few  days  after  birth  are  much  oftener  fatal 
than  those  occurring  later.  The  brain  has  less  power  of  re- 
sistance, and  the  processes  giving  rise  to  these  early  con- 
vulsions are  a  more  serious  menace  to  the  life  of  the  child. 

Secondly.  It  should  not  be  forgotten  that  convulsions  are 
so  much  more  frequent  during  the  first  two  years  of  life 
because  the  diseases  and  conditions  causing  convulsions  are 
most  frequent  at  this  period. 

We  must  take  into  account  an  injury  inflicted  upon  the 
brain  during  labor,  defective  brain  development,  dentition, 
the  acute  infectious  diseases,  and  almost  all  the  infantile 
cerebral  palsies  and  gastro-intestinal  disorders. 

Convulsions  may,  however,  occur  at  any  age  ;  but  since 
the  child's  brain  grows  less  irritable  the  older  it  grows,  a 
convulsion  occurring  later  in  life  often  signifies  more  serious 
trouble. 

The  frequency  of  convulsive  attacks  in  any  given  case 
will  also  depend  upon  the  nature  of  the  disease,  of  which 
it  is  merely  a  symptom.  In  the  case  of  convulsions  due  to 
dentition,  a  convulsion  or  a  series  of  convulsions  may  occur 
with  the  cutting  of  each  tooth ;  if  due  to  gastro-intestinal 
irritation,  convulsions  may  recur  until  the  condition  is  im- 
proved or  relieved.  In  acute  infectious  diseases  we  are 
apt  to  have  but  one  initial  convulsion,  or  at  most  two  or 
three  distinct  attacks ;  if  there  were  more  than  a  few  con- 
vulsions during  an  attack  of  an  acute  infectious  disease,  I 
should  suspect  some  cerebral  complication. 

It  is  a  fact,  important  and  well  worth  remembering,  that 


58  THE  NERVOUS  DISEASES    OF   CHILDREN. 

the  initial  convulsion  is  as  little  apt  to  be  repeated  as  is  the 
initial  rigor.  The  first  toxic  invasion,  and  not  the  fever, 
seems  to  cause  the  convulsions.  I  have  often  observed 
convulsions  with  relatively  low  temperatures  (1030  F.)  at  the 
onset,  without  any  repetition  of  the  same  during  the  course  of 
an  acute  disease,  even  though  the  temperature  reach  105 °  F. 
and  over.  I  do  not  mean  to  deny,  however,  that  high  fever 
alone  may  cause  convulsions,  but  it  is  not  a  common  cause. 

I  have  often  had  occasion  to  remark  that  repeated  con- 
vulsions are  characteristic  of  cortical  disease,  and  this  is 
borne  out  by  the  cerebral  diseases  of  childhood. 

In  the  majority  of  such  cases  the  convulsions  will  be  but 
one  of  a  series  of  symptoms ;  and  if  convulsions  occur  re- 
peatedly without  any  further  evidences  of  cerebral  disease, 
the  suspicion  of  true  epilepsy  must  be  entertained. 

Symptoms. — It  may  seem  odd  that  we  should  treat 
of  the  symptomatology  of  convulsions,  which  we  have 
termed  a  symptom  and  not  a  disease,  but  the  sequence  of 
events  during  a  convulsion  is  subject  to  great  variations, 
and  on  this  head  there  is  need  of  further  remark.  A  con- 
vulsion may  be  partial  or  general.  Partial  convulsions  in 
the  majority  of  the  cases  denote  organic  disease  of  the  brain, 
and  in  this  sense  may  be  more  serious  than  general  convul- 
sions ;  a  slight  convulsive  twitching  of  a  thumb  may  seem 
a  small  matter  indeed,  but  it  may  be  the  first  sign  of  a  cere- 
bral tumor  which  is  bound  to  be  fatal  before  long  ;  while,  as 
regards  danger  to  life,  a  severe  general  convulsion  may  be 
entirely  harmless.  Partial  convulsions  are  often  unilateral, 
and  sometimes  limited  to  a  single  member. 

The  cortical  centre  of  the  part  convulsed  is  the  chief  site 
of  the  lesion.  Partial  convulsions  often  become  general ;  it 
is  of  importance,  therefore,  to  inquire  whether  convulsions 
that  affect  all  parts  of  the  body  start  in  any  one  particular 
member,  or  whether  the  person  who  is  subject  to  general 
convulsions  ever  had  unilateral  partial  convulsions. 

All  the  phenomena  of  a  convulsive  seizure  need  not  to  be 
exhibited  in  each  attack.  We  may  have  convulsive  twitch- 
ings  without  loss  of  consciousness,  and  loss  of  conscious- 
ness without  twitching  movements.  I  have  clearly  in  mind 
a  child  about  three  months  old,  whom  I  had  reason  to  see 


CONVULSIONS— ECLAMPSIA    INFANTUM.  59 

some  nine  years  ago.  Without  any  known  cause  the  child 
was  in  a  "  faint  spell,"  during  which  time  the  skin  was  pale, 
and  the  pulse  became  slow  and  irregular.  Such  a  spell  oc- 
curred about  the  same  hour  on  successive  afternoons,  and 
later  on  the  child  had  several  each  day.  No  other  symptom 
could  be  made  out,  with  the  exception  of  a  distinct  enlarge- 
ment of  the  spleen.  The  attacks  were  evidently  malarial 
and  yielded  quickly  to  large  doses  of  quinine.  The  child 
has  had  no  convulsions  since. 

There  is  some  difference  of  opinion  among  authors  as 
to  whether  a  convulsive  attack  begins  with  clonic  or  tonic 
movements,  and  which  preponderate.  It  is  well  to  know 
that,  according  to  the  researches  of  Unverricht  and  others, 
tonic  and  clonic  convulsions  are  different  in  degree  and  not 
in  kind — a  tonic  convulsion  being  practically  a  rapid  suc- 
cession of  clonic  movements.  In  children  tonic  spasm  is 
supposed  to  precede  clonic  spasm,  and  in  this  respect  to 
differ  from  the  epileptic  paroxysm  of  later  life  ;  but  the  dis- 
tinction is  of  no  importance,  and,  moreover,  I  have  seen 
many  spasms  in  children  which  were  purely  clonic  from 
beginning  to  end. 

One  special  form  of  convulsive  seizure  in  children  de- 
serves special  mention.     This  is 

Laryngismus  Stridulus. — Cerebral  croup,  inward  con- 
vulsions, child-crowing,  etc.  The  many  synonyms  are  in- 
dicative of  the  frequency  of  this  condition,  which  occurs 
more  often  in  European  countries  than  here,  owing  prob- 
ably to  the  greater  spread  of  severe  forms  of  rickets  in 
England  and  on  the  Continent. 

In  its  simpler  form  laryngismus  stridulus  implies  merely 
a  spasm  of  the  glottis.  A  child  that  may  have  exhibited  an 
occasional  crowing,  croaking  noise,  is  seized  quite  suddenly 
with  severe  spasm  of  the  adductor  muscles ;  the  body  be- 
comes rigid,  the  head  is  drawn  back,  the  face  grows  pale 
and  then  livid.  In  a  few  seconds  the  spasm  relaxes,  a  deep 
inspiration  follows  with  a  hissing  sound,  and  all  is  over  for 
the  time  being,  except  that  the  child  presents  a  haggard, 
tired  look.  Several  such  spasms  may  follow  quickly  upon 
one  another.  After  the  attack  is  over  the  child  often 
vomits,  is  badly  frightened,  and  from  sheer  fatigue  falls 


DO  THE  NERVOUS  DISEASES   OF  CHILDREN. 

into  a  more  or  less  natural  sleep.  It  is  not  rare  to  have  as 
many  as  twenty  and  more  of  such  attacks  during  the  course 
of  twenty-four  hours ;  in  other  cases  single  attacks  follow 
each  other  at  much  longer  intervals.  It  is  quite  excep- 
tional, however,  to  have  but  a  single  spasmodic  attack. 

Other  convulsive  actions  are  apt  to  be  associated  with 
the  laryngeal  spasm.  The  diaphragm  and  other  respiratory 
muscles  are  often  convulsed ;  the  fingers  are  firmly  clenched, 
and  the  toes  are  in  extreme  plantar  flexion  ("carpopedal " 
spasm);  general  convulsive  movements,  and  a  total  loss  of 
consciousness  may  be  developed  before  the  seizure  is  over, 
proving  the  close  relationship  between  the  laryngeal  spasm 
and  general  convulsions.* 

Causation. — There  was  much  dispute  among  the  older 
authors  as  to  the  true  cause  of  laryngeal  spasm.  An  en- 
larged thymus  gland  pressing  upon  branches  of  the  vagus 
was  supposed  to  be  the  most  frequent  cause ;  but  this  has 
been  entirely  disproven  by  the  autopsies  of  Henoch  and 
others,  who  showed  that  there  was  no  correspondence  be- 
tween the  state  of  the  thymus  and  the  occurrence  of  "  in- 
ternal convulsions."  An  overpowering  weight  of  opinion  is 
in  favor  of  a  close  causal  relationship  between  rickets  and 
laryngismus  stridulus.  The  researches  of  Elsasser,  of  West, 
Gee,  Gay,  Henoch,  Jacobi,  Heubner,  and  many  others  have 
offered  substantial  proof  of  this  position.  Of  late  years 
there  has  been  a  disposition  to  discredit  this  theory.  Loos 
has  denied  the  influence  of  rickets,  while  endeavoring  to 
establish  a  relation  between  this  convulsive  disorder  and 
tetany.  To  my  mind  the  only  proper  relationship  between 
the  two  is  that  they  are  both  due,  safely  enough,  to  the 
influence  of  rickets.  Loos  has  been  severely  criticised,  and 
his  facts  have  been  disproven  by  Kassowitz,  whose  large 
experience  of  rickets  has  stood  him  in  good  stead. 

Rachitic  softening  of  the  skull  (craniotabes)  has  been 
supposed  by  Jacobi  and  others  to  be  the  active  cause  of 
laryngeal  spasm.  That  the  association  of  these  conditions 
is  unusually  frequent  cannot  be  doubted.  Kassowitz  states 
that  only  48  of  370  cases  of  laryngeal  spasm  did  not  present 

*  Children  suffering  from  laryngismus  stridulus  are  prone  to  the  ordinary  eclamptic 
fits,  thus  furnishing  further  evidence  of  the  close  relationship  between  the  two  conditions. 


CONVULSIONS— ECLAMPSIA    INFANTUM.  *         6 1 

marked  craniotabes ;  but  of  these  48  cases  47  presented 
some  other  decided  symptom  of  rickets.  The  fontanelles 
were  closed  in  only  4  of  these  370  cases,  although  110  of 
these  children  were  over  one  year  of  age.  When  rickets  is 
not  present  the  fontanelle  should  be  'closed  at  the  end  of 
the  first  year  (Kassowitz). 

But  is  a  softened  skull  the  direct  cause  of  laryngeal 
spasm  ?  Scarcely.  The  centres  for  vocal  movements  are 
far  away  from  that  part  of  the  brain  which  is  apt  to  be 
compressed  in  cases  of  craniotabes.  The  hyperaemic  condi- 
tion of  the  brain  in  rickets  is  of  a  piece  with  the  hyperaemic 
condition  of  other  structures  and  it  is  this  hyperaemia 
which  causes  an  unusual  irritability  of  the  centres  which 
would  not  under  ordinary  circumstances  "  discharge"  upon 
the  slightest  peripheral  irritation  (cold  air,  dentition,  slight 
gastro- disturbances).  Indeed  there  may  be  spontaneous 
discharge  of  these  centres  without  peripheral  irritation. 

Laryngeal  spasms  occur  most  frequently  between  the 
sixth  and  eighteenth  months,  the  very  period  during  which 
rickets  is  most  marked,  if  present,  though  it  occurs  often 
enough  up  to  the  age  of  three  years  and  later.  Patients  sub- 
ject to  attacks  are  most  apt  to  suffer  from  them  during  the 
coldest  months  of  the  year,  viz.,  January,  February,  and 
March. 

Much  has  been  made  of  the  supposed  relation  between 
laryngismus  stridulus  and  tetany.  Cheadle  believes  the 
two  to  be  identical.  Facial  contractility  may  be  present  in 
cases  of  laryngismus,  but  cases  of  laryngismus  in  which 
the  Trousseau  symptom  (spasm  induced  by  compression  of 
the  artery  in  a  limb)  can  be  elicited  during  the  interval  be- 
tween attacks  are  surely  rare. 

There  is  another  form  of  convulsive  seizure  in  children 
which  reminds  one  of  laryngismus.  Many  years  ago  a  child, 
aged  eighteen  months,  apparently  healthy,  was  brought  to 
me  that  would  hold  its  breath  in  many  a  crying  spell ;  after 
holding  its  breath  for  a  few  seconds  it  would  turn  blue,  and 
its  head  would  drop  forward  as  it  lost  consciousness.  After 
a  few  more  seconds  the  child  recovered  consciousness,  and 
all  was  well  again  until  the  next  crying  spell,  when  the 
same  sequence  of  symptoms  would  be  apt  to  occur.     Treat- 


62  THE  NERVOUS  DISEASES   OF   CHILDREN. 

merit  by  bromides  and  small  doses  of  chloral  put  an  end  to 
these  attacks  in  a  few  weeks. 

Diagnosis  of  Convulsions.  —  It  is  scarcely  credible 
that  any  one  who  has  ever  seen  a  convulsive  seizure  can 
mistake  it  for  anything  else.  And  yet  it  has  happened 
within  the  author's  experience  that  he  was  called  to  a  case 
of  supposed  tetany,  which  was,  however,  nothing  more  than 
an  ordinary  convulsive  attack. 

The  difficulties  of  diagnosis  that  arise  are  concerned  al- 
together with  the  possible  etiological  factor  in  a  given  case. 
The  physician  who  witnesses  a  convulsive  seizure  will  do 
well  to  be  reserved  in  giving  an  explanation  of  the  origin 
of  such  seizure  until  he  has  carefully  examined  into  the 
previous  history  of  the  case ;  and  if  he  does  this  he  will  be 
able  to  say  with  great  certainty  whether  the  convulsion  is 
due  to  some  reflex  irritation,  to  some  cerebral  or  spinal  dis- 
ease, or  whether  it  is  the  initial  symptom  of  an  acute  in- 
fectious disorder.  It  is  of  the  greatest  practical  importance, 
however,  to  be  able  to  say  distinctly  whether  the  convulsion 
is  the  expression  of  functional  disease  or  of  organic  disease 
of  the  brain.  General  convulsions  are,  in  the  vast  majority 
of  cases,  of  functional  origin.  Partial  convulsions  are,  as  a 
rule,  the  result  of  organic  disease ;  but  a  child  that  has  gen- 
eral convulsions  may  at  some  previous  stage  of  its  history 
have  exhibited  partial  seizures,  or  a  general  convulsion  may 
have  begun  in  a  strictly  localized  fashion.  The  part  first 
convulsed,  or  the  part  alone  convulsed,  indicates  that  the 
centre  in  the  brain  which  governs  the  movements  of  this 
special  part  of  the  body  is  the  chief  site  of  the  lesion.  A 
twitching  movement  of  the  thumb,  so  slight  that  many 
might  be  tempted  to  regard  it  as  a  trifling  nervousness,  indi- 
cates disease  in  or  near  the  centre  governing  the  movement 
of  the  thumb.  A  convulsive  seizure,  beginning  with  a 
twitching  of  the  eyelid,  with  the  drawing  up  of  one  corner 
of  the  mouth,  is  certain  to  be  the  result  of  disease  in  the 
respective  centres  of  these  parts. 

The  gravity  of  a  convulsive  seizure  will  depend  very 
much  upon  the  cause  of  the  attack/  In  general  terms  a  par- 
tial epilepsy  pointing  to  organic  lesions  of  the  brain  is  a  more 
serious  matter  than  a  general  epilepsy,  which  may  be  func- 


CONVULSIONS— ECLAMPSIA    INFANTUM.  63 

tional ;  but  if  a  general  convulsion  is  the  expression  of  a 
typical  epilepsy  about  to  be  developed,  its  importance  is  as 
grave  as  though  it  were  due  to  gross  disease  of  the  brain. 
Convulsions  occurring  at  the  onset  of  acute  diseases  are 
much  more  apt  to  pass  off  without  leaving  a  trace  behind 
them  than  are  those  convulsions  which  occur  during  the 
further  course  of  the  disease. 

There  is  considerable  difference  of  opinion  among  au- 
thors regarding  the  remote  dangers  of  convulsions.  Many 
are  inclined  to  believe  that  convulsions  are  always  sympto- 
matic, and  that  they  rarely  constitute  a  serious  danger  to 
the  child's  life  ;  but  others,  like  myself,  relying  chiefly  upon 
a  careful  study  of  infantile  cerebral  palsies  and  of  epileptic 
convulsions  in  the  adult,  are  confident  that  convulsions  are 
capable  of,  and  often  do  give  rise  to,  serious  disturbance. 
This  is  not  surprising  if  we  consider  that  extreme  cyanosis, 
with  an  accompanying  intense  hypersemic  condition  of 
the  brain,  occurs  during  the  acme  of  the  convulsion.  I 
have  myself  recorded  a  case  of  a  child  dying  in  an  epilep- 
tic convulsion,  in  which  at  the  post-mortem  table  a  wide- 
spread recent  subpial  hemorrhage  over  the  convexity  and 
at  the  base  was  found  to  have  been  the  immediate  cause  of 
death.  Eustace  Smith  refers  to  a  case  with  hemorrhage 
over  the  base,  evidently  the  effect  of  convulsions,  while 
Money  and  others  have  reported  cases  in  which  a  palsy, 
setting  in  after  a  convulsion,  was  evidently  due  to  hemor- 
rhage from  the  smaller  vessels  of  the  brain  coming  on  dur- 
ing, and  caused  by,  a  convulsive  seizure.  The  possibility 
of  such  occurrences  makes  a  convulsion  a  serious  matter. 
It  must  be  the  earnest  endeavor  of  the  physician  in  attend- 
ance on  the  child  to  bring  a  convulsive  seizure  to  an  end  as 
soon  as  possible.  The  longer  a  seizure  lasts,  the  more  fre- 
quently it  is  repeated,  the  greater  the  danger  becomes — if  not 
to  the  life  of  the  child,  at  least  to  its  future  mental  condition. 

Hence  it  follows  that  we  must  be  guarded  in  giving  a 
prognosis  of  the  child's  condition  after  the  seizure  is  over, 
the  majority  of  them  leaving  no  unfavorable  trace  upon  the 
child,  but  every  now  and  then  cases  occur  in  which  the 
convulsions  were  the  first  of  a  long  series  of  disasters.  I 
shall  never  forget  the  case  of  a  bright  child  who  had  a  con- 


64  THE  NERVOUS  DISEASES   OF   CHILDREN; 

vulsive  seizure  at  the  onset  of  chicken-pox ;  a  rapidly  de- 
veloping idiocy  was  the  result.  Seizures  occurring  in  the 
first  few  days  after  birth,  inasmuch  as  they  are  the  expres- 
sion of  serious  disease  in  the  brain,  give  a  particularly  un- 
favorable prognosis. 

Treatment. — The  first  object  of  treatment  should  be  to 
check  the  convulsion.  Many  remedies  will  have  been  ap- 
plied long  before  the  physician  has  arrived  at  the  bedside 
of  the  patient.  Hot  baths,  mustard  poultices  over  the  spine, 
the  abdomen,  to  the  soles  of  the  feet  are  still  in  vogue  and 
urged  not  only  by  knowing  mothers,  but  also  by  many  phy- 
sicians' (by  Meigs  and  Pepper,  for  instance) ;  but  all  these 
measures  are  to  be  deprecated,  for  they  imply  a  loss  of 
valuable  time.  If  a  convulsion  ends  while  the  child  is  in  a 
hot  bath  it  does  so  because  it  has  run  its  course. 

To  check  the  convulsion  inhalations  of  chloroform,  or  of 
chloroform  and  nitrite  of  amyl  in  equal  parts,  are  much  to 
be  preferred  to  other  forms  of  medication.  A  few  drops 
poured  on  a  handkerchief  and  held  to  the  nose  of  the 
child  are  generally  sufficient  to  diminish  the  severity  of  the 
convulsive  seizure,  and  the  nurse  or  physician  sitting  by 
can  keep  up  this  method  of  administering  the  drug  until 
the  tendency  to  convulsions  has  for  the  time  being  entirely 
disappeared.  Recognizing  the  seriousness  of  convulsions, 
the  physician  will  do  well  to  wait  until  all  danger  from 
further  attacks  has  passed.  After  the  acute  stage  is  over 
small  doses  of  morphia  (gr.  -^  to  ^)  or  of  opium,  according 
to  the  age  of  the  child,  may  be  given  ;  and  after  the  first 
day  has  been  passed  safely  I  should  advise  the  discontinu- 
ance of  the  opiates  and  the  substitution  of  small  doses  of 
bromide  (3  to  5  grs.)  several  times  a  day,  or  small  quantities 
of  chloral  hydrate  (gr.  v.  to  x.),  either  by  mouth  or  in  the 
form  of  a  rectal  enema. 

Absolute  quiet  is  the  first  essential  of  treatment.  If 
there  is  good  reason  to  believe  that  the  convulsive  seizure 
was  due  to  gastro-intestinal  irritation,  or  to  the  presence  of 
intestinal  parasites,  steps  should  be  taken  to  purge  the 
bowels  thoroughly,  say  by  the  use  of  castor-oil,  calomel, 
and  the  like,  or  by  appropriate  treatment  for  the  removal 
of  worms.     As  soon  as  the  cause  has  been  removed,  treat- 


CONVULSIONS—  ECLAMPSIA    INFANTUM. 

ment  by  small  doses  of  opium,  bromide,  or  chloral,  will  be 
in  order  for  at  least  a  week  or  ten  days  after  the  occurrence 
of  the  seizure. 

If  the  convulsive  attack  is  associated  with  fever,  there 
is  of  course  every  reason  for  giving  a  tepid  bath,  with  sub- 
sequent cool  sponging.  The  reduction  of  the  temperature 
will,  to  a  certain  extent,  diminish  the  probability  of  repeated 
attacks.  Should  a  child  be  seized  with  convulsions  while 
in  the  bath,  it  should  be  removed  at  once  and  made  to  in- 
hale chloroform  and  nitrite  of  amyl.  It  is  far  better  in 
the  cases  of  gastric  disturbance  to  evacuate  the  stomach 
through  the  bowels  than  to  attempt  to  give  emetics,  which 
of  course  cannot  be  readily  swallowed,  or  to  give  hypo- 
dermic injections  of  morphia  as  suggested  by  Steffen.  For 
to  cause  a  child  to  vomit  that  has  a  tendency  to  convulsions 
is  to  increase  the  danger  to  which  it  is  exposed. 

Cases  of  laryngismus  stridulus  can  be  treated  on  very 
much  the  same  principle  as  convulsions,  excepting  that  the 
child  should  not  be  allowed  to  remain  in  the  recumbent 
position  ;  it  should  be  carried  about,  and  if  breathing  is  inter- 
fered with,  forcible  means,  such  as  pulling  forward  of  the 
tongue,  slapping  of  the  chest  with  cold  and  wet  towels, 
should  be  resorted  to  in  order  to  bring  about  regular, 
rhythmical  breathing. 

If  the  convulsions  are  due  to  rickets,  very  active  treat- 
ment, looking  toward  the  improved  nutrition  of  the  child, 
will  be  necessary.  It  should  be  placed  upon  the  albumi- 
noids, and  only  small  quantities  of  the  cereals  allowed. 
Moreover,  in  these  cases,  Kassowitz's  plan  of  administering 
phosphorus  with  cod-liver  oil,  as  in  the  scrofulous  diathesis, 
is  of  greatest  importance.  Syrup  of  the  iodide  of  iron,  or 
any  of  the  many  preparations  of  iron  now  to  be  obtained  in 
the  market,  can  be  administered.  If  the  attack  is  the  be- 
ginning of  an  epilepsy,  no  time  should  be  lost  in  placing 
the  child  upon  the  regular  treatment  of  this  condition,  for 
the  details  of  which  the  student  is  referred  to  the  chapter 
on  Epilepsy.  In  the  cases  of  uraemic  and  other  toxic  con- 
vulsions, the  treatment  must  be  in  accordance  with  the 
nature  of  the  cause,  and  the  condition  is  to  be  treated  in 
every  respect  as  the  same  condition  would   be  treated  if 


66  THE  NERVOUS  DISEASES   OF   CHILDREN. 

it  occurred  in  the  adult.  If  the  attack  is  supposed  to  be 
due  to  cutting  teeth,  the  question  arises  whether  incision  of 
the  gums  is  apt  to  be  of  any  benefit.  There  has  been  much 
discussion  on  this  point,  and  yet  no  definite  conclusion  has 
been  reached ;  and  no  one,  so  far  as  I  have  been  able  to 
learn,  has  proved  that  cutting  the  gums  has  ever  injured 
the  child  or  impaired  its  future  health.  It  is  well  enough, 
therefore,  to  try  this  method,  and  personally  I  have  little 
doubt  that  a  temporary  relief  of  the  hyperaemic  condition 
of  the  gums  is  thus  afforded.  That  the  mere  incision  of 
the  gum,  as  Henoch  and  Lewis  suggest,  is  apt  to  increase 
the  peripheral  irritation,  I  can  scarcely  credit,  if  the  incision 
be  made  according  to  modern  surgical  practice. 


CHAPTER   III. 

EPILEPSY. 

Few  diseases  have  given  rise  to  so  much  discussion 
as  has  epilepsy,  the  "  morbus  sacer "  of  ancient  writers. 
Opinions  regarding-  its  cause  and  pathology  have  under- 
gone many  radical  changes.  The  causes  of  epilepsy  were 
by  most  writers  thought  to  be  obscure,  and  it  is  only  with- 
in very  recent  years  that  a  little  more  light  has  been  shed 
upon  the  morbid  processes  underlying  epileps)\  Some 
claim  that  epilepsy  is  never  a  disease  per  se,  always  a 
symptom,  while  others  are  inclined  to  regard  true  idio- 
pathic epilepsy  as  a  form  of  hereditary  disease. 

Whether  epilepsy  be  merely  a  symptom  or  a  distinct 
disease,  the  term  may  be  used  to  describe  a  state  in  which 
convulsive  seizures  occur  at  varying  intervals  of  time,  and 
in  which  there  is  no  special  cause  for  each  seizure.  Thus 
a  child  may  have  a  convulsive  fit  with  the  cutting  of  each 
tooth,  yet  we  cannot  say  that  it  has  epilepsy ;  but  if  that 
same  child,  after  dentition  and  its  attendant  troubles  have 
passed,  continues  to  have  fits  at  shorter  or  longer  intervals, 
the  child  is  surely  a  subject  of  epilepsy.  Fortunately,  how- 
ever, only  a  very  small  proportion  of  those  who  have  con- 
vulsions during  childhood  develop  true  epilepsy  in  later 
years.  Webber  reports  that  of  160  cases  of  epilepsy  only 
24  began  before  the  age  of  five. 

The  Symptoms  of  epilepsy  vary  with  the  character 
of  the  attacks.  Unfortunately  the  intervals  between  the 
attacks  are  not  marked  by  as  distinct  a  series  of  symp- 
toms as  is  the  case  in  tetany.  In  epilepsy  the  only  symp- 
toms that  we  can  find  in  the  interval  are  the  effects  of  biting 
the  tongue,  the  acne  resulting  from  the  bromide  treatment, 
and  the  general  stupor  and  indifference  which  are  as  often 
due  to  the  drugs  administered  as  to  the  disease  itself. 


68  THE  NERVOUS  DISEASES   OF  CHILDREN. 

Epileptic  attacks  can  be  divided  into  three  classes : 
i.  Major  attacks,  or  "grand  ma/." 

2.  Minor  attacks,  or  "petit  mal!' 

3.  Epileptic  equivalents. 

Some  patients  are  subject  to  major  and  minor  attacks, 
and  there  is  every  possible  gradation  between  the  graver 
and  lesser  seizures. 

The  "  grand  mal "  attacks  differ  but  very  little  from  the 
convulsive  seizures  as  described  in  the  preceding  chapter. 
It  will  be  sufficient  in  this  connection  to  enumerate  in  the 
order  of  their  importance  and  of  their  occurrence  the  chief 
symptoms  of  a  major  attack  of  epilepsv. 

1.  Prodromata,  generally  of  a  sensory  character.  At 
times  there  is  a  vasomotor  or  psychic  disturbance. 

2.  Initial  cry. 

3.  Loss  of  consciousness  (very  sudden). 

4.  Pupils  dilated  ;  no  reaction  ;  eyes  open  or  closed. 

5.  Tonic,  then  clonic  spasm  of  muscles  (unilateral,  partial, 
or  general). 

6.  Spasm  of  respiratory  muscles,  which  may  lead  to 
asphyxia. 

7.  Spasm  of  the  muscles  of  the  jaw  (biting  of  the  tongue, 
bloody  foam). 

8.  Spasm  relaxes,  movements  become  clonic  and  then 
intermittent. 

9.  Involuntary  passage  of  urine  or  of  fasces. 

10.  Gradual  recovery  of  consciousness,  followed  by  a 
prolonged  stupor  or  profound  sleep. 

n.  The  entire  seizure  lasts  only  a  few  minutes. 

12.  The  deep  reflexes  are  diminished  or  increased.  The 
Babinski  sign  may  be  present. 

In  "  grand  mal  "  attacks  most  of  these  symptoms  will  be 
present,  but  occasionally  we  meet  with  major  attacks  in 
which  there  are  no  distinct  prodromata  ;  in  which  the  initial 
cry  is  wanting ;  in  which  there  is  no  biting  of  the  tongue ; 
no  relaxation  of  the  sphincters.  The  most  constant  symp- 
toms are  the  loss  of  consciousness,  the  dilatation  of  the  pupils, 
the  spasm  of  the  muscles,  and  the  stupor  or  sleep  after  the 
convulsive  movements  have  ceased.  The  diagnosis  should 
rest  upon  the  presence  of  at  least  two  of  these  symptoms. 


EPILEPSY.  69 

The  prodromata  are  of  great  importance.  In  a  fair  pro- 
portion of  cases  the  patient  feels  that  an  attack  is  coming 
on.  A  vague  sensation  at  the  stomach,  a  feeling  of  numb- 
ness or  of  tingling  in  any  of  the  extremities,  are  by  far  the 
most  frequent  warning  signs.  In  other  cases  the  aura  con- 
sists of  slight  twitching  movements,  of  an  altered  psychic 
condition,  varying  from  a  general  restlessness,  or  irritability 
of  temper,  to  actual  maniacal  excitement.  In  many  instan- 
ces the  character  of  the  aura  points  to  the  involvement  of  a 
definite  portion  of  the  brain,  and  this  is  true  even  of  cases  of 
general  epilepsy  which  are  not  supposed  to  be  due  to  any 
gross  cerebral  lesion.  In  not  a  few  cases  temporary  aphasia 
marks  the  coming  on  of  an  attack. 

Auditory  symptoms  in  the  prodromal  stage  are  not  un- 
common. In  a  little  boy  whom  I  described  in  a  paper  pub- 
lished some  years  ago,  every  epileptic  attack  was  preceded 
by  a  hissing  noise  like  that  of  a  steam-engine  letting  off 
steam.  A  young  lady,  aged  twenty-five,  a  school-teacher, 
who  was  subject  to  epileptic  attacks  at  every  menstrual 
period,  is  in  awe  of  an  old  woman  whom  she  sees  regularly 
before  each  epileptic  seizure.  The  vision  of  a  ball  of  fire, 
of  the  colors  of  the  rainbow,  of  a  sudden  change  in  the  size 
of  objects,  is  a  common  premonitory  condition.  Still  an- 
other patient  perceives  a  very  foul  odor  during  the  aura; 
but  I  have  never  met  with  a  case  of  epilepsy  in  which  a 
pleasant  or  unpleasant  taste  preceded  an  epileptic  fit.  This 
would  seem  to  show  that  the  sense  of  taste  is  not  inde- 
pendent of  the  sense  of  smell,  although  some  authors  refer 
in  a  vague  manner  to  a  gustatory  aura. 

Patients  who  have  distinct  auras  are  on  the  whole 
more  fortunate  than  those  who  have  not,  for  they  are  able 
to  prepare  themselves  for  the  attack.  They  can  secure 
themselves  against  injury  during  the  attack  and  may  have 
time  to  apply  remedies  which  either  inhibit  or  shorten  the 
spells. 

The  convulsive  movements  should  be  carefully  studied 
in  each  case,  for  they  help  to  define  the  character  of  the 
epilepsy.  Generally  speaking,  universal  convulsions  point 
to  hereditary  epilepsy.  Partial  or  unilateral  epilepsy  is 
indicative    of   organic    cerebral   disease.     This   distinction 


JO  THE  NERVOUS  DISEASES   OF   CHILDREN. 

would  be  a  very  easy  one  were  it  not  for  the  fact  that 
partial  epilepsy  may  at  any  stage  of  the  disease,  and  at  any 
stage  of  an  attack,  become  general,  so  that  after  the  lapse 
of  time  the  convulsions  due  to  organic  disease  of  the  brain 
can  in  nowise  be  distinguished  from  those  which  are  pre- 
sumably hereditary  and  idiopathic.  It  is  of  the  utmost  im- 
portance, therefore,  in  making  the  diagnosis  of  epilepsy,  to 
inquire  into  the  past  history  of  the  case,  and  to  determine, 
either  by  personal  observation  or  by  close  questioning,  the 
exact  manner  of  onset  of  each  attack.  However  rapidly 
the  attack  may  become  generalized,  if  it  begins  each  time 
with  distinct  twitchings  of  the  thumb,  of  the  wrist,  even  of 
the  eyelid,  there  is  good  reason  to  remove  such  a  case  from 
the  category  of  hereditary  epilepsy  and  to  range  it  with 
those  due  to  organic  lesions.  The  attacks  may  come  on 
at  definite  hours  of  the  day  or  night.  In  "  nocturnal  "  epi- 
lepsy an  abrasion  of  the  tongue,  involuntary  micturition,  a 
tired  feeling  or  a  headache  in  the  morning  may  be  the  only 
evidences  of  an  attack.* 

The  typical  "  minor  "  attacks  consist  of  a  very  transitory 
loss  of  consciousness,  without  any  muscular  twitchings, 
without  the  peculiar  cry,  and  without  the  involuntary  pas- 
sage of  urine  and  fseces.  The  loss  of  consciousness  is  often 
so  slight  that  the  child  is  supposed  to  have  been  "  absent- 
minded  "  or  merely  "  faint."  Mothers  and  physicians, 
euphemistically  inclined,  are  apt  to  speak  of  these  attacks 
as  "  fainting  spells."  There  is  every  reason  to  suspect  epi- 
lepsy in  any  case  in  which  "  fainting  spells  "  occur  and  re- 
cur without  sufficient  exciting  cause.  In  some  instances  a 
sudden  lull  in  the  conversation  of  the  child,  a  momentary 
stare,  an  unexpected  stop  when  the  child  is  practising  upon 
an  instrument,  are  the  signs  of  petit  mal ;  a  change  in  the 
child's  mental  condition,  in  its  character  and  temperament 
(there  is  often  increased  irritability)  and  sometimes  a  mild 
form  of  mania,  reveal  the  serious  character  of  what  was 
supposed  to  be  a  mere  fainting  spell. 

Bourneville  has  found  a  slight  elevation  of  temperature 
during  major  attacks  (not  more  than  i°  F.);  in  the  status  epi- 
lepticus,  the  condition  of  continuous  spasms,  the  tempera- 

*  For  further  details  see  description  of  convulsive  attack. 


EPILEPSY.  J I 

ture  may  rise  several  degrees.  Thomsen  and  Oppenheim 
have  proved  the  existence  of  a  concentric  limitation  of 
vision,  and  a  diminution  of  general  sensibility  for  some  time 
after  an  epileptic  attack. 

The  term  "  procursive  epilepsy  "  has  been  applied  to 
attacks  in  which  the  patient  is  suddenly  impelled  to  run 
some  distance  forward,  or  backward  ;  this  may  be  a  pro- 
dromal symptom,  or  it  may  be  the  only  manifestation  of  an 
attack.     This  variety  is  very  rare  indeed. 

W"e  have  alluded  above  to  the  existence  of  epileptic 
equivalents.  Instead  of  having  a  typical  convulsive  seizure, 
the  child  passes  into  a  state  of  mental  confusion  in  which  it 
becomes  entirely  irresponsible  for,  and  unconscious  of,  its 
actions ;  it  may  also  pass  into  a  condition  of  trance  or  into 
an  acute  mania.  Prolonged  periods  of  double  conscious- 
ness are  rare  in  children ;  nor  have  I  seen  in  very  young 
persons  the  condition  of  narcolepsy  (sudden  falling  asleep) 
which  has  been  described  as  an  occasional  symptom  of 
epilepsy  in  the  adult.  These  "psychic  equivalents  "  may 
be  the  beginning  of  a  post-epileptic  insanity. 

The  mental  derangement  following  epileptic  attacks  is 
often  marked  by  violent  delirium.  Under  the  influence  of 
delusions  and  hallucinations,  the  patient  may  be  impelled 
to  deeds  of  cruelty.  Such  derangement  may  last  for  a  few 
hours  or  weeks,  or  it  may  become  chronic  and  lead  to  de- 
mentia. 

Causes. — In  considering  the  causes  of  epilepsy  we  shall 
take  up,  first,  causes  of  the  disease  itself,  and  secondly,  the 
causes  which  lead  to  individual  attacks.  That  epilepsy  is 
often  a  sad  heritage  can  scarcely  be  doubted  if  we  regard 
the  statistics  collected  by  Gowers,  who  finds  a  marked  pre- 
disposition in  two-thirds  of  1,450  cases.  The  inheritance 
seemed  to  him  to  come  more  freely  from  the  mother's  than 
from  the  father's  side.  It  is  also  beyond  dispute  that  epi- 
lepsy in  the  ancestry  is  not  the  only  predisposing  cause. 
Other  chronic  nervous  disorders  have  a  powerful  influence 
in  this  respect.  I  have  known  children  of  extremely  hys- 
terical, neurasthenic  mothers  to  develop  idiopathic  epilepsy. 
Chorea  in  the  mother  is  very  apt  to  lead  to  the  develop- 
ment of  true  epilepsy  in  the  child  ;  and,  on  the  other  hand, 


J  2  THE   NERVOUS  DISEASES    OF   CHILDREN. 

syphilis  and  alcoholism  in  the  father  exert  a  very  powerful 
influence  in  this  same  direction.* 

According  to  Mendel's  careful  studies  the  majority  of 
cases  of  hereditary  epilepsy  begin  before  the  age  of  twenty 
years;  but  up  to  the  age  of  forty  years  idiopathic  epilepsy 
may  manifest  itself.  The  same  author  proposes  to  call  the 
disease  "late  epilepsy,"  if  it  is  developed  after  the  age  of 
forty  years;  in  twenty-five  per  cent,  of  these  "late"  cases 
Mendel  found  distinct  hereditary  influences. 

Accepting  the  truth  of  all  these  statements,  I  wish  to 
impress  one  fact  upon  the  mind  of  the  student.  Cases  of 
hereditary  (idiopathic)  epilepsy  are  not  nearly  so  frequent 
as  they  are  supposed  to  be.  If  we  examine  carefully  into 
the  early  history  of  our  cases  we  shall  find  frequently 
that  the  child  has  either  sustained  some  severe  injury  to 
the  brain,  or  has  acquired  some  cerebral  lesion  early  in  life. 
The  paralysis  and  other  symptoms  which  were  due  to  the 
same  lesion  may  have  disappeared,  but  the  epilepsy  re- 
mains. I  was  led  to  this  conclusion  by  my  studies  of  infan- 
tile cerebral  palsies,  and  my  views  have  been  quoted  ap- 
provingly by  various  authors  (among  them  Freud)  who 
have  become  interested  in  this  subject. 

A  very  striking  instance  illustrative  of  this  is  that  of  a 
girl,  about  fifteen  years  old,  who  had  been  treated  by  many 
physicians  for  idiopathic  epilepsy  without  any  favorable 
result.  On  inquiring  into  the  early  history  of  the  case  I 
was  told  that  several  years  previously  the  girl  had  suffered 
a  slight  paralytic  stroke,  every  vestige  of  which  had  disap- 
peared with  the  exception  of  an  increase  of  the  reflexes  on 
one  side  of  the  body.  Testing  carefully  for  a  possible  dim- 
inution of  power,  I  found  distinct  traces  of  an  old  hemi- 
plegia. What  was  supposed  to  have  been  a  case  of  heredi- 
tary epilepsy  was  clearly  a  case  of  epilepsy  due  to  former 
cerebral  disease. 

The  number  of  cases  of  idiopathic  epilepsy  will  be  still 
further  diminished  if  we  exclude  from  the  number  those 
cases  in  which  there  is  evidence  of  defective  general  devel- 

*  Kowalewsky  has  insisted  on  the  importance  of  syphilis  in  hereditary  epilepsy, 
and  has  made  a  fanciful  distinction  between  epilepsy  due  to  paternal  or  maternal 
syphilis.     His  statements  need  corroboration. 


EPILEPSY.  73 

opment  of  the  brain;  and  those  associated  with  infantile 
cerebral  palsies;  for  the  paralysis  and  the  epilepsy  are  due 
to  organic  disease  of  the  brain.  The  cases  in  which  epi- 
lepsy is  developed  after  an  acute  infectious  disease  cannot 
be  considered  "idiopathic." 

The  most  characteristic  cases  of  idiopathic  epilepsy  are 
those  in  which  the  disease  is  first  noticed  between  the  tenth 
and  twentieth  years.  A  few  may  come  on  between  the 
twentieth  and  thirtieth,  but  those  that  occur  later  in  life,  or 
in  the  first  few  years  of  life,  should  be  very  carefully  anal- 
yzed before  the  diagnosis  of  hereditary  epilepsy  is  made. 

It  must  be  remembered  that  even  in  cases  with  dis- 
tinct hereditary  predisposition  some  other  exciting  cause 
must  be  present  to  develop  the  disease ;  thus  we  find  that 
masturbation,  disturbances  of  menstruation,  sexual  excesses, 
great  emotional  excitement,  a  blow  to  the  head,  are  causes 
which  lead  to  the  development  of  epilepsy  in  those  predis- 
posed.* 

Causes  which  Lead  to  Individual  Attacks. — In  a 
patient  suffering  from  epilepsy  any  interference  or  disturb- 
ance of  the  general  health  of  the  patient  may  bring  on  an 
epileptic  attack.  Indigestion  is  perhaps  the  most  powerful 
of  these  causes.  Herter  and  Smith,  in  an  excellent  research 
on  this  subject,  are  inclined  to  look  upon  putrefactive  proc- 
esses in  the  intestine  as  a  frequent  exciting  cause  of  epi- 
lepsy. They  do  not  believe  that  this  cause  would  be  suffi- 
cient to  produce  epilepsy  in  a  person  not  predisposed 
thereto.     The  same  may  be  said  of  eye  strain, f  of  urethral 

*  Reflex  epilepsies  are  described  by  many  authors  as  due  to  peripheral  injuries 
(hand  or  foot)  ;  in  such  cases  the  aura  begins  with  symptoms  referable  to  the  injured 
part.  A  permanent  epilepsy  is  rarely  established  in  this  way,  and  I  prefer  to  speak 
of  reflex  convulsions  rather  than  of  reflex  epilepsy. 

t  Ranney's  contributions  to  this  subject  are  put  forth  very  earnestly,  but  he  fails  to 
prove  more  than  that  the  eye-conditions  (errors  of  refraction,  muscular  insufficiencies, 
etc.),  may  cause  single  attacks  ;  but  they  are  surely  not  the  cause  of  the  epilepsy. 
Moreover,  every  neurologist  knows  that  by  treatment  directed  to  the  improvement  of 
the  eye-condition  the  attacks  may  be  suspended  temporarily,  but  the  disease  returns 
after  a  varying  lapse  of  time.  Ranney's  cases  are  for  the  most  part  reported  altogether 
too  early.  Hern  and  Dodd  have  given  but  little  support  to  Ranney's  (or  rather  Stevens's 
views),  and  their  statistics  are  open  to  the  same  criticism.  Instead  of  reporting  the 
"  cures  "  it  would  be  valuable  to  publish  the  later  history  of  all  cases  operated  upon. 
In  1904  the  author  has  again  reviewed  this  subject  and  has  seen  no  reason  to  change 
his  views. 


74  THE  NERVOUS  DISEASES   OF   CHILDREN. 

stricture,  of  narrow  prepuces,  and  of  laryngeal  irritation. 
I  doubt  whether  any  one  of  these  conditions  has  ever  been 
the  sole  cause  of  epilepsy,  though  I  am  willing  to  concede 
that  they  may  be  sufficient  to  produce  occasional  attacks  in 
persons  with  this  special  hereditary  taint. 

Improper  oxygenation  of  the  blood  is  another  frequent 
cause  of  attacks.  This  explains  why  so  many  epileptics 
have  their  seizures  in  crowded  court-rooms,  in  theatres,  and 
in  poorly  ventilated  bedrooms.  The  irritation  of  the  epi- 
leptic centres  in  the  lower  portion  of  the  brain  is  directly 
responsible  for  these  attacks.  After  allowing  for  all  these 
various  causes  we  are  compelled  to  admit  that  many  of  the 
epileptic  seizures  occur  at  more  or  less  regular  intervals, 
for  reasons  that  we  cannot  discern,  though  I  am  firmly 
convinced  that  the  more  carefully  we  regulate  the  life  and 
habits  of  the  epileptic  patient  the  fewer  attacks  he  will 
have.  It  is  very  largely  a  question  of  removing  all  excit- 
ing causes. 

A  few  powerful  drugs  have  been  known  to  cause  epi- 
lepsy. Heiman  described  a  number  of  cases  of  epilepsy  due 
to  poisoning  by  cocaine,  and  Tuczek  has  dwelt  upon  the 
potent  influence  of  antipyrin.  In  the  case  of  children 
cocaine  need  not  be  feared ;  but  the  careless  exhibition 
of  antipyrin  might  be  responsible  for  unpleasant  occur- 
rences. 

Gray  has  called  attention  to  the  frequent  occurrence 
of  epileptic  attacks  in  those  suffering  from  chorea  and  mi- 
graine. I  have  seen  several  patients,  in  advanced  years, 
in  whom  the  epilepsy  followed  upon  the  cessation  of  mi- 
graine. 

Diagnosis. — The  diagnosis  of  epilepsy  offers  but  little 
difficulty  if  the  character  of  the  individual  attacks  has  been 
clearly  made  out,  and  if  such  attacks  recur  at  more  or  less 
regular  intervals. 

The  epileptic  nature  of  a  seizure  is  determined  by  the 
presence  of  some  of  the  several  symptoms  which  go  to  make 
up  a  complete  attack.  The  chief  difficulty  will  be  experi- 
enced in  differentiating  between  an  epileptic  seizure  and  an 
ordinary  fainting  spell ;  also  between  epilepsy  and  hysteri- 
cal attacks. 


EPILEPSY. 


75 


The  characteristic  features  of  each  attack  will  be  found 
in  the  following  tables  : 


Epileptic  Attacks. 

Loss  of  consciousness  very  sudden. 
Warning  of  short  duration. 

Pupils   dilated ;   do   not   contract  to 

light. 
Pulse  unaltered. 
Tonic  and  clonic  spasms  in  various 

parts  of  the  body. 
Bloody  foam  at  mouth. 
Involuntary   passage    of    urine    and 

fasces. 
Prolonged  stupor  after  attack. 


Fainting  Spells. 

Loss  of  consciousness  gradual. 
Warning    of    some    minutes    before 

consciousness  is  lost. 
Pupils  contracted  or  unaltered. 

Pulse  feeble. 
No  spasms. 

No  evidence  of  biting  of  the  tongue. 
No  involuntary  passage  of  urine  or 

faeces  excepting  in  rare  instances. 
Recovery  prompt  after  attack. 


Epileptic  Attacks. 

Loss  of    consciousness   sudden   and 

absolute. 
Warning  of  short  duration. 

Pupils  dilated. 

Tonic  and  clonic  spasm. 


Eyes  turned  upward  and  inward. 

Involuntary    passage    of    urine   and 

fasces. 
Prolonged  stupor. 


Attacks  at  rarer  intervals. 
Duration  of  attack  short. 


Hysterical  Attacks'. 
Loss  of  consciousness  not  absolute. 

The  attack  often  preceded  by  emo- 
tional excitement. 

Pupils  not  dilated. 

Tonic  rigidity ;  exaggerated  conscious 
movements  ;  arching  of  back ;  ex- 
cessive noises. 

Eyes  staring,  not  turned,  sometimes 
closed. 

No  involuntary  passage  of  urine  or 
faeces. 

Recovery  gradual ;  no  stupor.  The 
patient  may  pass,  however,  into  a 
trance  condition. 

Attacks  may  be  frequently  repeated. 

Duration  of  attack  much  longer. 


The  distinction  between  organic  and  idiopathic  epilepsy 
can  easily  be  made  if  we  simply  call  to  mind  that  organic 
epilepsies  are,  as  a  rule,  partial,  if  not  unilateral.  Idio- 
pathic epilepsies  are  invariably  bilateral  or  general  in  their 
manifestations. 

If  organic  disease  is  suspected,  the  manner  of  onset  of  the 
individual  attack  should  be  carefully  determined,  for  partial 


j6  THE   NERVOUS  DISEASES    OF   CHILDREN, 

or  unilateral  manifestations  often  become  general  during 
the  attack.  Under  the  head  of  organic  epilepsies  we  must 
include  post-hemiplegic  epilepsy.  This  form  comes  on  with 
deplorable  frequency  after  the  paralytic  attacks  in  early 
life.  The  epileptic  movements  as  a  rule  affect  the  paral- 
yzed side,  but  after  the  disease  has  lasted  for  some  years 
the  child,  although  its  paralysis  is  unilateral,  is  prone  to 
have  general  epileptic  attacks.  Under  these  circumstances 
the  relation  between  the  general  epilepsy  and  the  hemi- 
plegic  form  of  paralysis  can  scarcely  be  doubted.  It  will 
not  be  amiss  to  insist  again  upon  the  fact  that  the  evidence 
of  an  existing  hemiplegia  may  be  so  slight  that  it  will  be 
overlooked  unless  specially  examined  for;  but  however 
slight  such  traces  may  be,  if  it  can  be  shown  that  the  epi- 
lepsy was  developed  after  the  onset  of  the  hemiplegia  there 
,  is  good  reason  to  suppose  that  both  the  paralysis  and  the 
epilepsy  are  due  to  the  same  organic  lesion.  I  consider  it 
a  safe  rule  in  epilepsy  beginning  in  childhood  to  examine 
particularly  for  exaggeration  of  the  reflexes ;  and  an  in- 
crease of  the  reflexes  in  one-half  of  the  body  is  quite  as  safe 
a  sign  of  a  preceding  hemiplegia  as  a  marked  paralysis  with 
contracture  would  be. 

The  sudden  onset  of  epileptic  movements  in  a  child  pre- 
viously healthy  should  lead  one  to  suspect  the  possibility  of 
intra-cranial  tumor ;  and  a  slight  weakness  of  the  parts  con- 
vulsed, a  possible  increase  of  the  deep  reflexes  in  that  same 
part,  the  presence  of  headaches  and  the  development  of 
optic  neuritis,  are  the  symptoms  that  we  must  look  for  in 
order  to  establish  or  to  discard  the  diagnosis  of  tumor. 

The  same  series  of  symptoms  may  occasionally  be  pres- 
ent in  cases  of  acquired  or  hereditary  syphilis  ;  if  so  they 
are  the  result  of  general  specific  infiltration  of  the  brain 
coverings,  or  of  the  development  of  gumma  together  with 
this  general  infiltration. 

Prognosis. — The  prognosis  of  epilepsy,  whether  of  the 
organic  or  idiopathic  order,  is  grave,  not  hopeless.  Some 
patients  with  undoubted  epilepsy  have  been  cured.  In 
many  others,  do  what  we  will,  the  attacks  recur  as  soon  as 
treatment  is  abandoned.  Painstaking  treatment  meets  its 
due  reward.     The  author's  records  contain  the  histories  of 


EPILEPSY.  17 

young  men  and  women  who  were  sorely  afflicted  in  early 
life  but  have  had  no  recurrence  of  epilepsy  for  periods 
varying  from  three  to  fifteen  years.  The  attacks  may  be 
inhibited  for  a  year  and  even  for  two  years,  but  may  return 
after  that  period  of  time  if  the  customary  treatment  has 
been  stopped.  In  this  respect  there  does  not  seem  to  be 
a  wide  difference  between  organic  and  idiopathic  epilepsy, 
except  that  in  the  former,  malignant  disease  may  lead  to 
an  early  fatal  termination.  The  frequent  development  of 
dementia,  idiocy,  or  of  epileptic  insanity,  makes  the  out- 
look gloomy  enough  in  many  instances.  In  spite  of  all 
recent  therapeutic  efforts  we  have  not  been  able,  however, 
to  accomplish  as  much  as  is  desirable  in  the  way  of  a  rad- 
ical cure  of  the  disease.  The  most  encouraging  hope  that 
we  have  is  that  in  the  beginning  of  the  disease  the  attacks 
may  be  due  to  special  conditions  which  do  not  necessarily 
imply  the  existence  of  hereditary  epilepsy.  The  fond  hope 
that  the  child  may  outgrow  the  tendency  to  epileptic  seiz- 
ures is  realized  every  now  and  then. 

Pathological  Anatomy.— Our  knowledge  of  the  mor- 
bid changes  in  epilepsy  is  very  incomplete.  Meynert  laid 
great  stress  upon  induration  of  the  cornu  amonis.  Chronic 
hydrocephalus  has  been  found  in  many  cases,  but  this  can 
hardiy  be  considered  a  cause  of  epilepsy,  for  if  it  signifies 
anything  it  merely  implies  that  the  same  process  which  led 
to  the  development  of  hydrocephalus  also  caused  the  epi- 
lepsy. 

It  is  much  more  probable  that  the  morbid  changes  of 
true  epilepsy  will  be  revealed  through  a  study  of  the  corti- 
cal changes  which  give  rise  to  a  localized  epilepsy  in  cases 
of  serious  injury  to  the  brain,  and  of  epilepsy  associated 
with  infantile  hemiplegia.  In  these  conditions  the  initial 
stages  of  a  morbid  process*  leading  to  epilepsy  can  best  be 
studied ;  and  it  is  possible,  if  not  probable,  that  general  epi- 
lepsy has  such  a  beginning  at  a  time  and  in  a  part  of  the 
brain  unknown  to  us.  Marie,  Fere  and  Chaslin  have  led  the 
way  in  these  studies,  and  report  the  discovery  of  a  neuroglia 

*  For  other  morbid  processes  associated  with  epilepsy  see  chapters  on  Cerebral  Pal- 
sies and  on  Idiocy. 


78 


THE   NERVOUS  DISEASES   OF   CHILDREN. 


sclerosis  which  they  consider  to  be  the  cause  of  epilepsy — 
such  sclerosis  starting  from  a  focus  of  disease.  These  find- 
ings have  been  confirmed  more  recently  by  Alzheimer. 

Van  Gieson  examined  corti- 
cal tissue,  removed  by  McBur- 
ney,  from    two   cases  of  Starr. 
He    demonstrated    most    satis- 
factorily   the    changes    in    the 
large  pyramidal  cells  of  the  cor- 
tex and  in  the  neuroglia.      He 
that  "  the  ganglion  cells 
iffected   by  a  series  of  de* 
ive    changes,    which    in 
lost  advanced  stages  re- 
an  almost  complete  dis- 

FlG.  41.— Diffuse  Neuroglia  Sclerosis      solution  of  the  Cell,  and  yet  this 

degeneration  is  not  extensive 
enough  to  involve  the  cells  so 
universally  as  to  interfere  with  their  topographical  distri- 
bution." 

Some  of  the  degenerative  changes  of  the  cells  will  be 

evident  in  the  figure  below  taken  from  Van  Gieson's  article. 

There  is  a  decided  change — a  true  hyperplasia — in  the 


of  the    Cortex   in    Epilepsy   (after 
Chaslin). 


Fig.  42.— Various  Phases  of  the  Earlier  Stages  of  the  Degeneration  of  the  Ganglion 
Cells.  The  thin  lines  enclosing  the  cells  w  and  u  represent  the  pericellular  spaces  ; 
the  cells  x  and  y  show  the  earliest  stages,  w  and  s  later  stages,  and  k  shows  the 
ultimate  destruction  of  the  whole  of  the  ganglion-cell  body,  leaving  nothing  but  the 
nucleus  lying  in  an  empty  space.     (Van  Gieson.) 


EPILEPSY.  79 

neuroglia  tissue  ;  clusters  of  an  increased  number  of  very 
young  and  seemingly  proliferating  neuroglia  cells  are  vis- 
ible in  the  vicinity  of  the  small  pyramids. 

These  findings  were  very  much  the  same  in  two  cases  : 
in  one  the  condition  was  due  to  a  foreign  body,  and  in  the 
other  an  old  cicatrix  seems  to  have  induced  a  similar  path- 
ological state  in  the  neighboring  tissue.  To  appreciate 
such  researches  at  their  true  worth,  it  is  important  to  note 
that  they  have  reference  to  the  early  conditions  only.  What 
the  nature  of  the  secondary  changes  is,  and  how  these  are 


. )       u,  •  ■  ,*■•*..     jn  ^S^SSs  - 


vizznj 


Fig.  43.— A  Group  of  More  Mature  Neuroglia  Cells  in  the  Layer  of  Small  Pyramids. 

(Van  Gieson. ) 

developed  from  the  original  forms  of  disease  is  a  subject  for 
further  study.  Sailer  has  demonstrated  the  occurrence  of 
an  hypertrophic  nodular  gliosis  in  cases  of  epilepsy  associ- 
ated with  dementia.  Voisin  questions  whether  this  condi- 
tion will  be  found  in  genuine  epilepsy. 

Pathology.— The  pathology  of  the  epileptic  attack  is 
identical  with  that  of  convulsive  seizures  in  general.  It  is 
not  necessary  therefore  to  repeat  in  this  chapter  what  was 
said  in  the  chapter  on  convulsions.  The  only  question  of 
importance  is  whether  in  the  diseased  brain  the  convulsive 
centre  on  the  floor  of  the  fourth  ventricle  plays  as  impor- 
tant a  role  as  it  does  in  physiological  experiments.  Bins- 
wanger  found  that  electrical  stimulation  of  the  lower  half 
of  the  floor  of  the  fourth  ventricle  in  animals  would  pro- 
duce tetanoid  attacks  or  spasm  in  the  limbs  and  the  trunk, 
but  he  did  not  succeed  in  causing  an  ordinary  epileptic  at- 


80  THE  NERVOUS  DISEASES   OF   CHILDREN. 

tack.  There  can  be  very  little  doubt  that  in  man  an  epi- 
leptic attack  is  in  the  vast  majority  of  cases  the  result  of 
cortical  irritation.  The  character  of  the  sensory  aurae  is 
evidence  of  this,  and  so  is  the  observation  made  by  Oebeke 
and  Gowers,  that  epileptic  attacks  cease  after  a  lesion  in 
the  internal  capsule.* 

As  for  the  pathology  of  the  disease  itself,  opinions  have 
undergone  a  very  marked  change  within  the  last  few  years, 
owing  to  the  rather  startling  suggestion  of  Pierre  Marie, 
that  epilepsy  is  often  of  infectious  origin.  Haig,  Voisin, 
Fere  and  others  advocate  the  theory  of  auto-intoxication, 
believing  this  to  be  due  to  the  retention  of  toxic  substances 
in  the  blood.  Urine  voided  after  an  attack  has  been  found 
to  be  more  toxic  than  urine  voided  before  the  attack.  Let 
us  not  forget  that  a  focus  of  disease,  due  to  purely  me- 
chanical causes,  may  be  the  beginning  of  general  changes 
throughout  the  brain,  which  are  responsible  for  the  per- 
manent establishment  of  epilepsy. 

Treatment. — In  discussing  the  treatment  of  epilepsy  it 
is  best  to  proceed  on  the  theory  that  the  attack  is  due  to 
over-action  of  the  cortical  cells.  Gowers  explains  the  influ- 
ence of  the  bromides  by  stating  that  "  if  we  regard  the 
morbid  state  in  epilepsy  as  an  instability  in  the  resistance 
of  the  nerve-cells,  it  seems  probable  that  the  effect  of  the 
bromide  is  to  increase  the  stability  of  that  resistance." 
Whatever  the  explanation  may  be,  we  have  up  to  this  time 
found  no  drugs  that  can  in  any  sense  be  considered  proper 
substitutes  for  the  bromides.f 

It  is  certain  that  very  few  cases  of  epilepsy  have  been 
permanently  cured  by  the  administration  of  the  bromides ; 
but  unquestionably  they  serve  an  admirable  purpose  in 
checking  the  number  of  attacks  and  in  diminishing  their 

*  Horsley  pleads  strongly  for  the  invariable  cortical  origin  of  the  epileptic  attack, 
and  believes  that  during  the  attack  the  cortex  is  in  a  congested  rather  than  an  anaemic 
condition.  It  will  help  us  very  little  to  say  that  the  attacks  are  due  to  toxine  poi- 
soning. This  may  or  may  not  be  true  ;  but  in  every  case  the  most  important  ques- 
tion to  settle  is,  why  should  certain  individuals  be  prone  to  attacks  and  others  not, 
while  these  same  toxines  from  intestinal  putrefaction,  for  instance,  are  present  in 
hundreds  of  others  who  never  have  an  epileptic  seizure. 

t  A  recent  writer  explains  the  action  of  the  bromides  by  their  power  to  diminish 
the  effect  of  the  toxines  circulating  in  the  body.  If  toxines  and  bromides  were  in- 
jected into  the  veins  of  an  animal  at  one  and  the  same  time,  the  result  was  less 
severe  than  if  the  toxine  alone  was  injected. 


EPILEPSY.  8 1 

severity.  To  accomplish  this  end  the  bromide  salts  should 
be  administered  according  to  a  definite  plan.  It  has  been 
my  practice  to  give  preference  to  the  bromide  of  sodium, 
which  I  employ,  according  to  the  age  of  the  patient,  in  ten 
or  fifteen  grain  doses,  three  times  a  day.  If  given  in  a 
wineglassful  of  (alkaline)  water  after  meals,  the  gastric 
functions  will  not  be  seriously  impaired.  Erlenmeyer  sug- 
gested, some  years  ago,  that  a  combination  of  the  three 
salts — the  bromide  of  sodium,  the  bromide  of  ammonium, 
and  the  bromide  of  potassium — would  answer  far  better 
than  the  exhibition  of  any  one  of  these  salts  singly.  He 
went  so  far  as  to  claim  that  if  a  single  salt  had  produced 
acne,  that  that  acne  would  disappear  upon  the  administra- 
tion of  the  three  salts  combined.  I  have  given  this  method 
a  fair  trial,  but  have  not  been  able  to  convince  myself  of 
the  truth  of  Erlenmeyer's  claim.  The  bromides  should  be 
pushed  in  every  case  to  the  point  of  tolerance,  and  until 
the  attacks  have  been  successfully  diminished  or  inhibited. 
The  loss  of  the  palate  reflex  is  evidence  that  the  patient 
has  become  thoroughly  brominized,  but  in  children  intol- 
erance to  the  drug  is  very  apt  to  be  established  long  before 
this  point  has  been  reached. 

The  bromides  can  be  administered  in  divided  doses,  two 
or  three  times  a  day  ;  but  if  there  is  no  good  reason  to 
give  the  drug  during  the  day,  there  is  a  special  advantage 
in  giving  the  entire  daily  dose  shortly  before  bedtime.  The 
stupefying  effect  of  the  drug  is  not  felt  as  distinctly  as 
when  it  is  administered  during  the  daytime,  and  the  sound 
sleep  that  ensues  is  an  additional  advantage  to  the  patient. 
In  the  case  of  nocturnal  attacks  the  medicine  should  be 
given  before  going  to  bed,  and  at  no  other  time.  In  his 
various  admirable  lectures  on  the  treatment  of  certain  func- 
tional neuroses  Seguin  advocated  the  plan  of  giving  the 
bromides  on  very  much  the  same  principle  that  one  would 
adopt  in  giving  quinine  in  malaria.  In  other  words,  the 
bromides  should  be  given  according  to  the  periodicity  of 
the  attacks,  giving  little  in  the  interval  and  increasing  the 
dose  very  much  at  the  time  when  an  attack  is  expected ;  or 
if  the  attacks  are  frequently  repeated,  to  give  the  drug  four 
to  five  hours  prior  to  the  time  at  which  the  seizures  occur. 


82  THE  NERVOUS  DISEASES   OF   CHILDREN. 

I  can  subscribe  thoroughly  to  this  recommendation,  as  it  has 
given  me  admirable  results  in  the  treatment  of  epileptic 
attacks  in  children.  If  the  tendency  to  attacks  has  been 
overcome,  the  drug  should  be  continued  for  a  period  of  at 
least  one  year  after  the  last  attack.  The  dose  may  be  di- 
minished gradually,  but  it  is  wise  to  keep  the  patient  in  a 
state  of  mild  brominism.  The  good  effect  of  the  drug  can  be 
maintained  by  giving,  in  conjunction  with  the  bromide,  five 
to  ten  grains  of  chloral  hydrate,  a  few  minims  of  the  tincture 
of  digitalis,  or  of  the  tincture  of  belladonna.  Bromipin  in 
daily  doses  of  ten  to  twenty-five  grammes  has  been  useful. 
Innumerable  other  drugs  have  been  suggested  ;  among 
these  I  will  mention  cannabis  indica,  which  is  particularly 
effective  in  cases  of  epilepsy  with  chronic  headaches.  The 
oxide  and  lactate  of  zinc  have  been  freely  recommended, 
but  I  have  seen  no  beneficial  results.  The  administration 
of  iron  and  arsenic  is  thoroughly  rational;  no  doubt  the 
restorative  effect  of  these  substances  upon  the  blood  is  the 
indirect  cause  of  improvement  in  the  epileptic  condition. 
Some  years  ago  Gowers  advised  the  use  of  fifteen  to  thirty 
grains  of  borax  several  times  daily.  A  fair  trial  has  been 
given  this  drug  in  my  clinic  and  in  private  practice,  but  I 
cannot  claim  more  for  it  than  for  dozens  of  other  methods.* 
Collins  has  pleaded  in  favor  of  nitroglycerin  (in  cases  with 
a  vaso-motor  aura),  but  condemns  simulo,  osmic  acid  and 
hydrastis.  Flechsig  has  warmly  urged  a  combination  of 
opium  and  bromides.  Opium  is  given  for  six  weeks  ;  begin- 
ning with  one-half  to  one  grain  ;  the  dose  is  increased  gradu- 
ally until  the  patient  takes  eight,  ten,  or  fifteen  grains  daily  ; 
then  it  is  stopped  suddenly,  and  the  bromides  in  thirty- 
grain  doses,  four  times  daily,  are  substituted ;  after  some 
time  the  dose  is  diminished  to  five  or  ten  grains  per  day.  I 
was  ready  to  adopt  this  suggestion,  as  I  had  for  many  years 
been  using  codeine  together  with  the  bromide  salts.  In 
children  the  doses  must  be  somewhat  altered,  but  the  plan 
of  treatment,  according  to  my  own  experience,  deserves 
further  trial. f 

*  Toulouse  and  Richet  have  advised  the  withdrawal  of  chlorides.  Non-salted  foods 
are  to  be  given.  The  bromides  will  be  more  effective  if  less  salt  is  taken  into  the 
system. 

t  Bechterew  has  advised  a  combination  of  bromides  and  Adonis  vernalis. 


kPILEPSY.  83 

The  treatment  of  epilepsy  is  by  no  means  exhausted  by 
the  recital  of  a  few  medicinal  agents.  Success  depends 
upon  other  factors.  The  greatest  possible  attention  should 
be  paid  to  the  details  of  the  patient's  daily  life  ;  regularity 
in  the  hours  of  sleep,  in  the  hours  of  meals,  and  careful 
dieting,  are  essential  to  proper  treatment.*  I  place  my  epi- 
leptic patients  upon  a  general  mixed  diet,  including  small 
quantities  of  albuminoids  and  a  liberal  vegetable  diet,  but 
I  forbid  all  indigestible  articles  of  diet.  I  also  limit  the 
amount  of  cereals,  and  try  to  check  intestinal  fermentation 
as  far  as  possible,  being  moved  to  this  by  the  result  of  Her- 
ter's  researches.  Pastry  and  sweets  of  all  sorts  are  strictly 
prohibited.  Fresh  air  at  all  times,  and  particularly  at  night, 
should  be  insisted  upon.  Freedom  from  all  emotional  ex- 
citement is  another  recommendation  that  should  not  be 
overlooked.  Marked  ocular  defects  and  other  peripheral 
conditions  which  may  cause  an  epileptic  attack  are  to  be 
treated. 

Older  patients  who  have  distinct  warning  of  their  epi- 
leptic attacks  should  be  provided  either  with  the  pearls  of 
nitrite  of  amyl,  which  they  can  break  up  and  inhale  at  short 
notice,  or  else  they  can  be  given  a  mixture  of  equal  parts  of 
chloroform  and  nitrite  of  amyl,  which  can  be  carried  in  a 
small  phial  and  can  be  poured  upon  a  handkerchief  for 
purposes  of  inhalation  as  soon  as  the  attack  is  signalled. 
In  the  case  of  partial  epileptic  attacks  of  definite  onset  a 
ligature  applied  around  the  part  first  convulsed,  to  be  tight- 
ened quickly  as  soon  as  the  warning  comes,  has  been  rec- 
ommended by  Hughlings  Jackson,  and  is  serviceable  in 
some  cases. 

Surgical  Treatment.— The  surgical  treatment  of  epi- 
lepsy has  attracted  great  attention  during  the  past  fifteen 
years  or  more,  ever  since  it  has  been  known  that  the  motor 
centres  in  the  cortex  can  be  safely  and  easily  reached 
by  the  surgeon's  knife.  Surgical  interference  should  be 
considered  only  in  cases  of  partial  epilepsy  pointing  to  a 
definite  focus  of  disease  as  the  starting-point  of  the  entire 
trouble.     Nothing  seems  easier  than  to  expose  the  centre 

*  In  recent  years  rectal  olive-oil  injections,  given  at  frequent  intervals,  have  proved 
to  be  of  great  service. 


84  THE  NERVOUS  DISEASES   OF   CHILDREN. 

of  the  part  first  convulsed  in  an  epileptic  attack,  to  remove 
this  centre,  and  thus  to  remove  the  seat  of  disease,  but,  un- 
fortunately, our  ardent  hopes  in  this  matter  have  not  been 
realized. 

Some  years  ago  Dr.  Gerster  and  myself  reported  the 
results  of  operation  in  ten  cases  of  epilepsy.  The  cases 
were  carefully  selected,  and  if  surgical  operation  could 
have  been  of  avail  in  any  case  of  epilepsy,  it  would  have 
been  so  in  those  we  selected  for  operation ;  we  were  bound 
to  admit  that  in  our  experience  some  slight  improve- 
ment followed,  but  also  that  not  a  single  absolute  cure 
had  been  effected.  Since  that  article  was  written  my  ex- 
perience has  been  fully  doubled,  and  the  conclusions  reached 
are  practically  the  same.  An  analysis  of  the  cases  reported 
by  Starr,  which  included  my  own  cases,  does  not  justify  one 
in  taking  a  more  hopeful  view  of  this  question.  I  have 
referred  in  former  writings  to  the  reason  for  this  disap- 
pointment. After  an  initial  injury  to  the  brain,  months, 
and  sometimes  years,  elapse  before  the  epileptic  habit  is 
established.  In  about  the  same  length  of  time  a  general 
cerebral  sclerosis  has  been  developed  in  connection  with 
the  original  focus  of  disease.  We  may  remove  the  orig- 
inal focus,  but  the  general  sclerosis  remains  and  will  act 
as  a  constant  irritant  upon  the  remaining  epileptic  centres. 

On  the  other  hand,  it  would  be  hasty  to  conclude  that  the 
surgeon's  skill  can  be  dispensed  with  in  these  cases.*  The 
aim  should  be  to  watch  carefully  for  the  development  of 
epilepsy,  and  to  remove  injured  tissue  at  the  earliest  pos- 
sible date.  More  than  this,  I  consider  it  important  not  to 
await  the  actual  development  of  epilepsy ;  and  if  the  brain 
has  sustained  any  considerable  injury  to  remove  the  in- 
jured tissues,  which,  if  allowed  to  remain,  constitute  a  per- 
manent menace  to  the  future  health  of  the  patient.  We 
shall  be  able  to  prevent  the  development  of  epilepsy  very 
much  more  readily  than  we  can  cure  it  if  once  established. 

The  surgical  procedures  in  vogue  at  the  present  day  for 
the  cure  of  epilepsy  are  trephining  and  excision  of  diseased 
tissues.     Trephining  has  been  practised  for  centuries.     It 

*  The  author  wishes  to  direct  particular  attention  to  the  excellent  work  done  by 
Kocher. 


EPILEPSY.  85 

is  a  method  that  entails  no  special  danger  upon  the  life  of 
the  patient  operated  upon,  and  can  therefore  be  tried  with 
perfect  impunity  except  in  very  young  children.  White, 
of  Philadelphia,  and  others  believe  that  its  only  effect  is  the 
same  as  that  of  any  operative  procedure  ;  but  I  am  inclined 
to  think  that  if  it  has  any  good  effect  it  is  in  the  way  of 
relieving  increased  pressure,  and  that  increased  pressure  is 
more  frequently  a  real  factor  in  epilepsy  than  is  generally 
supposed,  on  account  of  the  frequency  of  cystic  formations 
and  of  the  increased  ventricular  fluid  in  a  very  large  pro- 
portion of  the  cases.  Excision  of  tissue  would  seem  to  be 
a  rational  procedure  in  the  earlier  stages  of  epilepsy  de- 
veloped after  skull  injury,  or  in  connection  with  early  cere- 
bral disease ;  but  the  paralysis  which  so  easily  results  from 
the  excision  of  the  motor  centres  militates  somewhat  against 
the  advisability  of  this  practice,  although  according  to 
Dana's  and  my  own  experience  such  paralysis  disappears 
after  a  few  weeks.  In  cases  in  which  the  epileptic  attack 
is  preceded  by  sensory  or  psychic  phenomena,  a  removal 
of  the  auditory  or  visual  centres,  for  example,  would  be  a 
serious  matter  indeed,  if  not  entirely  unjustifiable.  Many 
a  patient  would  prefer  the  occasional  repetition  of  an  epi- 
leptic attack  to  a  permanent  loss  of  hearing  or  a  permanent 
impairment  of  sight. 

The  only  other*  surgical  procedure  which  has  been 
suggested  for  the  cure  of  epilepsy  is  the  ligature  of  one  or 
both  vertebrals,  as  was  first  done  by  Dr.  Alexander,  of  Liv- 
erpool. The  dangers  of  this  operation  are  so  great,  and 
the  results  so  uncertain,  that  it  scarcely  merits  serious  Con- 
sideration. 

*  I  do  not  consider  it  necessary  to  refer  to  the  surgical  treatment  of  the  eye-muscles 
for  the  cure  of  epilepsy  except  utterly  to  condemn  the  practice.  As  I  am  treating  of 
epilepsy  in  children,  I  need  not  discuss  oophorectomy  and  similar  procedures. 


CHAPTER   IV. 

HYSTERIA. 

True  hysteria  is  a  relatively  rare  condition  in  adults.* 
This  may  seem  a  very  heterodox  statement  to  those  who 
have  been  ready  to  apply  the  term  hysteria  to  many  affec- 
tions of  the  nervous  system  which  could  not  be  attributed 
to  organic  lesions.  Those  who  are  advised  of  the  recent 
conception  of  hysteria  will  not  hesitate  to  indorse  the  au- 
thor's opinion.  The  determination  of  the  chief  symptoms 
of  true  hysteria,  and  the  separation  of  this  disease  from 
many  other  functional  disturbances  which  were  once  classed 
with  it,  have  constituted  a  signal  achievement  in  modern 
neurology.  We  have  at  last  succeeded  in  establishing  a 
number  of  symptoms,  chiefly  of  a  sensory  character,  which, 
when  present,  enable  one  to  make  the  diagnosis  of  hysteria 
with  a  great  degree  of  certainty,  and  which  do  away  with 
the  necessity  of  reaching  this  diagnosis  by  the  process  of 
exclusion.  As  the  physician's  knowledge  of  nervous  dis- 
eases increases,  the  diagnosis  of  hysteria  is  made  more  and 
more  infrequently.  In  the  author's  very  large  clinical  ser- 
vice at  the  New  York  Polyclinic  the  diagnosis  of  hysteria 
was  one  of  the  rarest  of  all  diagnoses  made.  In  other  insti- 
tutions with  which  the  writer  is  connected,  particularly  in 
the  Montefiore  Home  for  Chronic  Invalids,  hysteria  is  found 
to  be  much  more  frequent,  especially  among  the  foreign- 
born  inmates.  If  hysteria  is  a  rare  disease  in  the  adult,  it  is 
still  rarer  in  the  child,  but  when  it  does  occur  it  is  an  im- 
portant factor  in  the  future  life  of  the  child.  Jolly,  Ferrier, 
Oppenheim,  and  others  report  cases  of  hysteria  occurring 
as  early  as  the  second  or  third  year  of  life. 

*  We  see  much  less  of  true  hysteria  in  this  country  than  in  Europe  ;  it  is  also  far  less 
common  in  England  and  in  Germany  than  in  France  and  Russia.  Broadly  speaking, 
the  Anglo-Saxon  race  is  less  prone  to  the  development  of  hysteria  than  the  other  races 
represented  in  our  population. 


HYSTERIA.  87 

In  the  development  of  hysteria,  hereditary  influences 
are  the  most  potent  factors.  Charcot  and  his  followers  rule 
out  all  other  causes.  Given  an  hereditary  predisposition, 
mental  overexertion,  fright,  emotional  excitement,  exhaust- 
ing diseases,  trauma,  masturbation,  early  sexual  irritability 
(Freud)  may  be  the  contributing  causes. 

English  and  American  authors  have  had  verv  little  to 
say  upon  the  subject.  In  the  large  hand-book  of  Gerhardt, 
Jolly  has  treated  the  subject  in  a  very  satisfactory  manner, 
and  in  American  Cyclopaedias  and  Systems  of  Medicine 
the  subject  has  received  careful  attention  at  the  hands  of 
Mills  and  Lloyd.  Henoch  has  written  a  very  full  chapter 
on  the  subject,  but  includes  under  this  heading  many  forms 
of  disease  which  are  more  properly  designated  by  other 
names.  The  French  authors  have  naturally  had  much  to 
say  on  the  subject,  and  they  include  so  man)-  different  forms 
under  this  heading  that  one  is  puzzled  to  know  whether 
there  is  any  form  of  nervous  or  mental  disease  that  is  not 
in  some  way  related  to  hysteria  or  influenced  by  it. 

The  symptoms  of  the  disease  are  truly  protean  in  char- 
acter. No  one  person,  fortunately,  ever  exhibits  even  a 
majority  of  them.  Hysteria  in  one  person  is  very  different 
from  the  disease  as  it  is  exhibited  in  others.  It  is  difficult, 
therefore,  to  give  any  one  clinical  picture  of  the  entire  dis- 
ease. It  will  be  best  to  take  up  the  chief  symptoms  as  they 
occur  independently  of  other  diseases,  or  as  they  are  known 
to  complicate  other  functional  and  organic  diseases  of  the 
nervous  system.  Hysteria  is  characterized  by  symptoms 
which  point  to  a  defect  in  the  various  systems  and  organs 
of  the  body.     Thus  we  have  : 

1.  Psychic  manifestations. 

2.  Motor  manifestations. 

3.  Sensory  manifestations  and  vasomotor  disturbances. 
1.  Psychic  or  Mental  Hysteria. — Properly  speaking, 

every  case  of  hysteria  would  come  under  this  heading. 
For,  if  there  is  any  one  feature  that  distinguishes  hysteria 
from  other  diseases,  it  is  the  defect  of  will-power  and  the 
excess  of  the  emotional  faculties.  "  I  can't "  is  the  pet 
phrase  of  all  hysterical  subjects,  or  still  better,  "  I  will  not." 
It  is  not  so  much  a  direct  lack  of  power  to  exert  the  will, 


88  THE  NERVOUS  DISEASES   OF   CHILDREN. 

as  a  tendency  to  exert  it  in  perverse  fashion.  This  peculiar 
mental  condition  is  easily  recognized,  and  often  leads  to  the 
diagnosis  of  hysteria  in  persons  who  have  other  symptoms 
pointing  to  a  much  more  serious  disease.  But  aside  from 
this  general  hysterical  state  of  mind  there  are  other  mental 
conditions  which  are  very  typical  of  hysteria.  The  most 
pronounced  psychic  form  of  hysteria,  as  observed  in  chil- 
dren, is  that  known  as  hysterical  mania.  Under  great  men- 
tal strain  or  excitement  a  child  is  seized  with  a  crying  or 
laughing  spell,  after  which  it  passes  into  a  state  of  nervous 
excitement  in  which,  as  in  a  little  girl,  aged  eleven,  under  my 
observation,  the  child  becomes  violent,  attempts  to  strike 
others,  to  injure  herself,  and  to  tear  her  clothes  from  the 
body,  and  to  do  all  possible  injury  irrespective  of  conse- 
quences. 

Another  condition,  sometimes  classed  under  the  heading 
of  hysteria,  is  observed  in  young  girls,  particularly  at  the 
age  of  puberty,  and  often  ends  in  a  condition  of  marked 
acute  mania.  1  have  seen  a  number  of  such  cases  in  girls 
who  were  over-ambitious  and  eager  to  pass  their  school  ex- 
aminations. They  would  keep  up  under  the  excitement  of 
the  examination,  but  immediately  thereafter,  whether  suc- 
cessful or  not,  would  become  irritable,  excitable,  sleepless, 
would  have  laughing  and  crying  spells  by  turns,  would  re- 
fuse to  take  nourishment,  and  eventually  would  either  re- 
cover under  proper  treatment,  or  else  pass  into  a  condition 
of  typical  acute  mania  with  absolute  loss  of  reason,  with  in- 
tense excitement,  and  confused  delirium.  There  would  be 
no  reason  to  consider  these  cases  in  this  connection  if  they 
did  not  occur  in  children  who  have  shown  either  a  marked 
nervous  predisposition,  or  who  have  exhibited  hysterical 
symptoms  of  one  kind  or  another  at  previous  periods. 
Such  children,  if  subjected  to  severe  strain  or  severe  emo- 
tional excitement,  are  very  apt  to  pass  into  a  condition  of 
hysterical  mania. 

Other  mental  conditions  which  cannot  well  be  separated 
from  hysteria,  are  those  known  as  hystero-epilepsy,  cata- 
lepsy, hysterical  trance,  and  the  like.  Of  these,  hystero- 
epilepsy  is  by  far  the  most  important,  and  the  gravest  dis- 
order.    Mills  is  inclined  to  consider  this  form  of  disease 


HYSTERIA.  89 

very  rare  among  children.  In  this  he  is  undoubtedly  correct ; 
but  it  has  been  my  good  fortune  to  see  a  number  of  classical 
instances  of  this  special  form  in  children,  and  the  account 
I  give  is  based  entirely  upon  personal  impressions.  The 
gravity  of  hystero-epilepsy  is  increased  by  the  fact  that 
the  children  so  afflicted  are  found  most  frequently  in  de- 
generate families.  Insanity,  epilepsy,  chorea,  chronic  alco- 
holism, are  the  predisposing  conditions  in  the  ancestors  of 
those  who  suffer  from  hystero-epilepsy.  In  one  family  I 
have  during  a  period  of  ten  years  treated  the  mother  for 
severe  hysteria,  a  daughter  at  the  age  of  nine  for  hysteri- 
cal convulsions,  and  another  son  and  daughter  for  typical 
hystero-epilepsy. 

On  account  of  this  close  relationship  to  true  epilepsy, 
cases  of  hystero-epilepsy  cannot  be  regarded  with  the  in- 
difference which  many  physicians  still  display  toward  hys- 
terical subjects.  In  many  cases  it  is  difficult  to  decide 
whether  the  attacks  as  they  occur  are  more  hysterical  or 
more  epileptic  ;  and  of  the  attacks  occurring  in  one  individ- 
ual some  may  be  typically  hysterical,  while  others  may  be 
typically  epileptic  ;  and  there  is  some  danger  in  every  case 
of  severe  hystero-epilepsy  that  with  the  progress  of  years 
the  hysterical  symptoms  may  vanish  and  true  epileptic  at- 
tacks may  occur.  It  is  of  the  utmost  importance,  before 
passing  judgment  upon  any  case,  to  determine  whether  the 
attacks  are  epileptic  or  hystero-epileptic. 

2.  Motor  Manifestations. — Every  variety  of  clonic 
and  tonic  movements  occur  in  hysterical  subjects.  Many 
of  these  will  be  understood  best  by  referring  in  detail  to  a 
few  cases  of  typical  hystero-epilepsy. 

Some  seven  years  ago  I  was  called  to  see  a  lad,  then 
thirteen  years  old,  who  had  been  seized  with  violent  con- 
vulsions during  the  night.  I  had  been  informed  that  these 
convulsions  were  preceded  by  great  emotional  excitement, 
caused  by  a  severe  upbraiding  which  the  boy  received  for 
his  misbehavior.  After  this  little  domestic  scene  was  over 
the  boy  went  to  bed,  and  was  seized  with  the  first  attack. 
During  the  attack  he  gave  a  shrill  cry  ;  then  began  to  bark 
like  a  dog,  snapping  at  everyone  who  approached  him,  and 
would  pass  through  the  most  severe  contortions,  touching 


90  THE  NERVOUS  DISEASES   OF   CHILDREN. 

the  bed  at  times  only  with  the  head  and  heels,  the  back  be- 
ing- deeply  arched  as  in  the  famous  drawings  of  Richer 
representing  this  condition  in  women.  After  this  the  most 
violent  jactations  of  the  entire  body  occurred.  During 
these  convulsive  movements  he  would  snap,  bark,  and  bite, 
then  he  would  suddenly  give  a  leap  to  the  foot  of  the  bed — 
almost  tumbling  out  of  bed — yet  he  always  saved  himself  in 
time.  This  whole  performance  would  last  about  two  or  two 
and  a  half  minutes,  then  thorough  relaxation  of  all  the  mus- 
cles would  take  place,  he  would  fall  back  exhausted,  and 
would  then  regain  consciousness.  During  the  attack  there 
was  no  evidence  of  consciousness,  at  least  no  impression 
could  be  made  upon  him  through  any  of  the  special  senses. 
The  patient  often  had  as  many  as  two  hundred  such  attacks 
in  the  course  of  twenty-four  hours ;  he  was  sleepless,  refused 
food,  and  became  very  much  emaciated.  After  careful  nurs- 
ing for  a  period  of  six  weeks  the  attacks  gradually  lessened, 
and  eventually  he  recovered  entirely  ;  but  he  has  shown  since 
a  deficiency  in  his  moral  and  mental  make-up,  and  although 
he  has  been  cured  of  the  hystero-epilepsy,  it  was  found 
necessary  to  place  him  in  a  reformatory.  This  last  fact  is 
of  some  interest  as  showing  the  relation  which  hystero- 
epilepsy  bears  to  degeneration  of  all  the  mental  and  moral 
faculties. 

Another  case  was  that  of  a  young  girl,  aged  fourteen, 
who  was  much  worried  over  the  fear  that  she  and  the  family 
would  starve,  as  the  father  had  daily  drummed  into  the 
child's  ears  the  necessity  of  economy  and  the  difficulties  of 
supporting  a  family  in  hard  times.  The  young  girl,  the 
daughter  of  a  very  hysterical  and  emotional  mother,  took 
the  warning  to  heart,  and  for  the  first  time  in  her  life  de- 
veloped typical  hystero-epileptic  attacks.  Without  warning 
she  would  fall  from  a  chair,  from  a  sofa,  or  even  on  the 
street  would  pass  suddenly  into  a  condition  of  apparent  un- 
consciousness. Wherever  she  lay  she  would  pass  through 
all  sorts  of  contortions,  would  shriek,  now  and  then  would 
give  agonizing  yells,  and  would  finally  end  up  by  a  pro- 
nounced crying  spell.  At  other  times  she  would  become 
violent,  tearing  her  own  clothes,  the  bed-linen,  and  every- 
thing that  was  within  her  reach  in  the  room.     These  spells 


HYSTERIA. 


91 


55 


92 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


were  repeated  very  frequently  during  a  period  of  three 
weeks,  and  then  gradually  subsided.  During  all  these  at- 
tacks the  movements  seemed  to  be  more  or  less  purposive, 
and  yet  there  was  good  reason  to  think,  and  the  girl  later 
on  confessed,  that  she  was  absolutely  unconscious  of  what 
she  was  doing  at  the  time.  These  two  cases  will  suffice  as 
a  general  indication  of  the  common  form  of  hystero-epilep- 
tic  attacks. 

The  distinction  between  the  attacks  of  epilepsy  and  of 
hystero-epilepsy  is  brought  out  in  the  following  table : 


Epilepsy. 

Aura  frequent. 

No  distinct  cause  for  each  attack. 

Onset  sudden. 

Initial  cry. 

Eyes  open  or  closed ;  pupils  often 
dilated  widely  ;  do  not  react ;  roll- 
ing of  eyes  upward  and  inward. 

Tonic  and  clonic  convulsions. 


Involuntary  micturition  or  defeca- 
tion. 

Duration  of  attack  only  a  few  min- 
utes. 


The  patient  often  injures  himself. 

Biting  of  the  tongue. 
After  cessation  of  clonic  movements, 
stupOr  or  somnolence. 


Hystero-Epilepsy. 

No  aura ;  but, 

Some  emotional  excitement,  direct 
cause  of  attack. 

Onset  sudden  or  gradual. 

Noises  of  all  sorts  during  attack. 

Eyes  turned  up ;  pupils-  normal ; 
often  ecstatic  expression  of  coun- 
tenance. 

Either  tonic  rigidity  of  muscles  or 
extravagant  movements  (some- 
times purposive). 

No  impairment  of  vesical  and  rectal 
reflexes. 

Attacks  last  much  longer,  followed 
by  a  condition  of  trance,  or  else 
patient  recovers  consciousness  as 
soon  as  convulsive  movements 
cease. 

The  patient  falls  softly,  without  per- 
sonal injury. 

Biting  of  the  tongue  rare.  ' 

Patient  may  pass  into  condition  of 
trance,  or  may  exhibit  signs  of  fa- 
tigue ;  recovery  often  rapid. 


In  addition  to  the  typical  attacks  of  hystero-epilepsy 
there  are  other  forms  of  motor  disturbance  due  to  hysteria. 
The  hystero-epileptic  attack,  while  it  is  the  gravest,  is  in  fact 
much  rarer  than  the  ordinary  hysterical  convulsion.  The 
latter  is  characterized  by  a  temporary  and  imperfect  loss 
of  consciousness  ;  by  irregular,  though  sometimes  violent, 
twitchings  of  all  extremities,   sometimes  by  the   repetition 


HYSTERIA.  93 

of  one  special  form  of  convulsive  movement,  such  as  re- 
traction of  the  head.  When  these  various  convulsive  move- 
ments are  over,  the  muscles  pass  into  a  state  of  tonic  rigid- 
ity, after  which  rapid  recovery  takes  place.  The  entire 
episode  is  wound  up  by  a  fit  of  crying  or  laughing,  or  by 
some  other  expression  of  an  emotional  character.  Some  of 
the  patients  pass  into  a  condition  of  melancholy  which  may 
last  for  hours,  or  into  a  state  of  exaltation  and  even  of  ec- 
stasy. Thjs  is  as  true  of  the  hysterical  attacks  as  they  oc- 
cur in  children  as  it  is  of  those  that  occur  in  the  adult. 
These  attacks  can  be  distinguished  from  the  hystero-epilep- 
tic  and  from  epileptic  seizures  by  the  incomplete  loss  of 
consciousness,  by  the  absence  of  all  regular  rhythmical 
convulsive  movements,  and  by  the  fact  that  there  is  neither 
biting  of  the  tongue,  nor  involuntary  micturition,  nor  any 
of  those  symptoms  which  are  often  associated  with  hystero- 
epileptic  and  epileptic  attacks.  Other  hysterical  attacks 
consist  of  spasm  of  the  head  and  neck  muscles,  the  well- . 
known  spasm  of  the  oesophagus  which  gives  rise  to  the 
sensation  known  as  globus  hystericus,  or  to  difficulties  in 
deglutition  if  the  spasm  affects  the  lower  part  of  the  gul- 
let. This  oesophageal  spasm  which  occurs  in  hysterical 
and  otherwise  neurotic  children  is  a  symptom  which  has 
not  met  with  the  appreciation  which  it  deserves.  If  a 
sound  is  passed  into  such  a  gullet  it  will  be  seen  that  there 
is  an  actual  muscular  spasm  at  the  point  of  constriction, 
and  that  it  requires  considerable  patience,  and  sometimes  a 
little  force,  to  overcome  the  contraction ;  but  the  age  of  the 
child  and  the  fact  that  the  constriction  easily  disappears,  to 
return  again  under  the  slightest  emotional  excitement,  is 
sufficient  to  remove  any  fear  of  the  constriction  being  due 
to  an  organic  cause.  In  this  same  category  we  may  place 
spasmodic  movements  of  the  diaphragm,  of  which  singultus 
is  the  common  manifestation.  This  special  phenomenon  is 
much  more  frequent  in  later  periods  of  life  than  during  the 
period  of  childhood;  but  1  have  had  under  observation  an 
entire  family  afflicted  with  this  special  form  of  hysteria 
who  upon  the  slightest  provocation  would  exhibit  this 
symptom.  On  one  occasion  I  examined  the  mother,  in  the 
presence  of  two  daughters,   respectively   twelve   and    ten 


94  THE  NERVOUS  DISEASES   OF   CHILDREN. 

years  of  age.  Under  the  excitement  of  the  examination 
the  mother  was  seized  with  severe  singultus,  and  before  I 
had  completed  the  examination  both  the  daughters  were 
favoring  me  with  a  similar  exhibition.  The  scene  would 
have  been  a  comical  one  if  it  had  not  brought  home  to  me 
the  powerful  influence  of  example  and  suggestion,  if  not  of 
heredity,  in  such  cases. 

Spasm  of  the  bladder,  increased  peristaltic  action  of 
the  stomach  and  bowels  resulting  in  diarrhoea,  upon  the 
least  emotional  excitement,  fits  of  crying  or  laughing,  of 
sneezing  and  of  coughing,  are  the  various  forms  of  hyster- 
ical spasm  met  with  in  children,  as  well  as  in  persons  of 
more  advanced  age.  If  the  hysterical  seizure,  or  better 
said,  the  hysterical  spasms,  assume  a  more  extravagant 
character,  and  if  it  affects  a  larger  number  of  the  groups  of 
muscles  that  generally  act  in  unison  with  one  another,  we 
may  have  that  peculiar  condition  which  is  known  as  chorea 
major.  In  this  form  the  jactations  are  often  severe  and  ex- 
hausting. The  child  or  young  person  assumes  the  most  im- 
possible positions,  often  suggesting  the  intention  of  dra- 
matic effect.  From  this  condition  it  is  but  a  short  step  to 
a  condition  of  mild  tonic  contracture,  the  limbs  retaining 
any  position  which  may  be  given  them.  This  state  of  cata- 
lepsy is  very  often  witnessed  in  conjunction  with  chorea 
major,  sometimes  independently  of  it,  but  it  is  a  mistake 
to  assume  that  catalepsy  occurs  exclusively  in  conjunction 
with  hysteria.  It  occurs  in  connection  with  various  forms 
of  mental  derangement,  and  a  condition  not  unlike  cata- 
lepsy is  met  with  in  some  forms  of  infantile  cerebral  paraly- 
sis. 

In  hysteria  the  muscles  of  the  body  are  not  only  subject 
to  convulsive  movements  but  also  to  absolute  paralysis. 
Every  part  of  the  body,  including  the  ocular  muscles,  may 
be  the  seat  of  such  palsy,  but  it  is  most  frequently  mani- 
fested in  the  extremities,  in  the  tongue,  and  in  the  vocal 
cords  (hysterical  aphonia).  In  some  instances  there  is  no 
absolute  paralysis  of  any  one  muscle  or  group  of  muscles, 
but  certain  functions  are  paralyzed.  The  best  known  exam- 
ple of  this  is  the  form  which  French  neurologists  have  termed 
astasia-abasia.     A  person  thus  afflicted  is  neither  able  to 


H1TSTERIA.  95 

stand  nor  to  walk,  but  is  perfectly  well  able  to  use  all  mus- 
cles while  lying  on  the  back.  Many  have  claimed  that  this 
is  simply  due  to  a  psychic  condition  dominated  by  the 
idea  that  standing  or  walking  is  impossible.  This  is  in  all 
probability  the  proper  interpretation  ;  but  the  point  that 
concerns  us  here  is  that  it  rarely,  if  ever,  occurs  except  in 
those  who  exhibit  other  symptoms  of  hysteria. 

Hysterical  aphonia  is  very  frequent  in  children,  and  par- 
ticularly during  the  period  of  development.  The  hysteri- 
cal character  of  the  aphonia  is  easily  recognized  by  the  fact 
that  (as  the  laryngoscope  reveals)  there  is  no  organic  cause 
for  the  loss  of  voice,  and  that  the  single  attacks  come  on 
very  suddenly,  as  a  rule,  in  the  wake  of  some  nervous  ex- 
citement, and  disappear  as  rapidly  as  they  have  appeared. 
I  have  had  young  girls  come  to  my  consulting-room  who 
would  not  be  able  to  speak  above  a  whisper,  and  it  has  al- 
ways been  a  special  pleasure  to  have  them  sing  the  entire 
scale  before  leaving  the  room.  A  strong  faradic  brush  ap- 
plied to  the  neck  over  the  trachea  is  the  most  persuasive 
master  in  these  cases.  Hysterical  mutism,  an  absolute  loss 
of  speech,  is  generally  the  result  of  severe  emotional  ex- 
citement. In  some  instances  it  is  associated  with  delusions, 
and  with  other  symptoms  of  mental  derangement. 

Other  forms  of  hysterical  paralysis,  ocular  palsies,  for 
instance,  are  not  easy  to  diagnosticate,  and  in  order  to  dis- 
tinguish them  from  paralysis  due  to  organic  causes  the 
physician  must  have  all  neurological  facts  and  doctrines  at 
his  command.  It  is  indeed  one  of  the  most  difficult  problems 
in  neurology  to  distinguish  hysterical  from  organic  forms 
of  paralysis,  but  this  distinction  can  be  made  safely  enough 
if  the  following  points  are  kept  in  mind  :  Hysterical  paraly- 
sis does  not  as  a  rule  adhere  to  anatomical  distribution,  with 
the  one  exception  that  an  hysterical  hemiplegia  may  be  quite 
as  complete  as  any  hemiplegia  due  to  an  organic  disease. 
But  I  have  not  yet  met  with  a  single  case  of  hysterical  hemi- 
plegia in  a  child  that  has  in  any  way  suggested,  even  for  a 
moment,  the  possibility  of  a  hemiplegia  due  to  an  organic 
lesion  in  the  brain.  In  hysterical  paralysis  the  paralysis,  as 
well  as  the  anaesthesia  which  is  associated  with  it,  is  apt  to 
be  regional ;  thus  we  have  a  paralysis  of  the  hand,  or  a  pa- 


g6  THE  NERVOUS  DISEASES   OF  CHILDREN. 

ralysis  of  hand  and  forearm,  or  paralysis  of  an  entire  extrem- 
ity, with  an  anaesthesia  that,  as  a  rule,  develops  in  proportion 
to  the  paralysis.  This  association  of  regional  anaesthesia 
and  regional  paralysis  is,  to  my  mind,  very  characteristic  of 
hysteria.     Hysterical  contractures  are  not  infrequent. 

From  cerebral  palsy,  hysterical  paralysis  can  be  distin- 
guished by  the  fact  that  it  is  not  accompanied  by  increased 
reflexes ;  and  by  the  lack  of  marked  sensory  changes  in  the 
majority  of  cases  of  brain  paralysis.  The  deep  reflexes  may 
be  lively  in  cases  of  hysterical  paralysis,  but  I  have  never 
seen  them  markedly  exaggerated,  nor  are  they  accompanied 
by  the  spasticity  and  tonic  contractures  which  are  of  such 
frequent  occurrence  in  the  case  of  paralysis  due  to  brain 
disease.  The  presence  of  ankle  clonus  was  at  one  time  sup- 
posed to  prove  the  non-hysterical  character  of  an  affection ; 
this  may  be  true  in  the  majority  of  cases,  but  since  I  have 
met  with  the  presence  of  ankle  clonus  in  some  hysterical, 
and  even  in  neurasthenic,  affections,  I  cannot  attach  much 
importance  to  this  one  point.  The  electrical  reactions,  it 
should  be  remembered,  are  normal  both  in  hysterical  and 
in  cerebral  diseases ;  we  cannot  therefore  utilize  them  in  any 
way  in  making  the  differential  diagnosis. 

If  the  question  arises  whether  paralysis  of  the  upper  or 
lower  extremities  (more  frequently  of  the  latter)  is  due  to 
spinal  disease,  or  whether  it  be  purely  hysterical,  the  diag- 
nosis should  be  based  upon  the  absence  of  vesical  and  rec- 
tal symptoms  in  a  case  of  hysterical  paralysis,  though  to  be 
sure  there  are  many  cases  of  spinal  disease  too  in  which 
these  are  not  affected.  Retention  of  urine  may  occur  in 
cases  of  hysteria,  but  it  is  only  of  short  duration  as  a  rule, 
and  is  rarely  accompanied  by  the  symptoms  of  cystitis,  as  is 
the  case  in  a  large  number  of  spinal  palsies.  In  many  cases 
of  spinal  and  peripheral  nerve  disease  the  electrical  reac- 
tions are  altered,  and  the  reaction  of  degeneration  may  be 
present.  This  is  not  the  case  in  hysteria ;  but  these  elec- 
trical reactions  are  normal  also  in  all  those  cases  of  spinal 
disease  which  are  due  to  disease  of  the  lateral  columns  and 
not  of  the  anterior  or  central  gray  matter. 

All  these  points  of  differential  diagnosis  may  in  some 
cases  prove  insufficient,  and  the  diagnosis  of  hysteria  or  of 


HYSTERIA.  97 

organic  disease  of  the  nervous  system  will  depend  upon  the 
general  agreement  or  disagreement  of  symptoms.  If  the 
physician  is  aware  that  flaccid  paralysis  of  a  single  group 
of  muscles,  or  of  one  or  more  extremities,  is  generally  asso- 
ciated with  changes  in  the  electrical  reactions,  and  with  loss 
of  reflexes  in  case  these  symptoms  are  due  to  organic  dis- 
ease, and  if  he  remembers,  furthermore,  that  spastic  forms 
of  paralysis  are  associated  with  increased  reflexes,  with  nor- 
mal electrical  reactions,  and  with  normal  sensations,  he  will 
have  little  or  no  difficulty  in  arriving  at  a  correct  diagnosis. 
Hysterical  paralysis  is  subject,  moreover,  to  greater  changes 
than  the  organic  palsies  are.  While  under  certain  condi- 
tions the  very  persistence  of  the  symptoms  for  a  long  period 
of  time,  followed  by  a  sudden  change,  is  extremely  char- 
acteristic of  hysterical  paralysis,  the  diagnosis  becomes 
still  more  certain  if  the  hysterical  paralysis  is  associated 
with  other  symptoms,  such  as  are  known  to  be  purely  hys- 
terical. I  refer  particularly  to  the  presence  of  rigidity, 
anaesthesia,  or  typical  and  complete  hemianaesthesia,  and  to 
the  occurrence  of  major  or  minor  hysterical  attacks  ;  but  the 
difficulties  are  still  further  increased  by  a  fact  to  which 
Seguin  called  particular  attention  some  years  ago,  that 
hysterical  paralysis  or  hysterical  symptoms  often  compli- 
cate organic  disease. 

French  authors  have  reported  a  number  of  cases  of  extreme  progressive 
wasting  of  muscles  in  hysteria.  Hirt  has  recorded  a  case  in  a  young  girl,  so 
astounding  in  the  rapidity  and  degree  of  development  of  the  atrophy  that  it 
almost  challenges  belief. 

3.  Sensory  Symptoms. — From  the  preceding  pages  it  is 
evident  that  the  sensory  symptoms  often  enable  one  to  make 
a  positive  diagnosis  of  the  hysterical  character  of  the  affec- 
tion when  other  symptoms  would  leave  some  room  for 
doubt.  Every  form  of  sensation  may  be  affected,  and  it 
may  either  be  exaggerated  or  dimished.  Hysterical 
hyperesthesia  and  hysterical  anaesthesia  are  of  common 
occurrence.  If  hyperaesthesia  exist  there  is  an  unusual 
sensitiveness  to  the  slightest  touch  or  to  the  slightest  pain- 
ful impression.  This  hyperaesthesia  is  most  prominently 
developed  in  the  region  of  the  ovaries,  in  the  skin  over  the 
7 


98  THE  NERVOUS  DISEASES   OF   CHILDREN. 

spinal  column,  each  spinous  process  being  so  sensitive  to 
touch  that  the  patient  cries  out  from  pain,  however  light 
the  touch  may  be.  The  hyperaesthetic  areas,  if  stimulated, 
are  very  apt  to  cause  distinct  hysterical  attacks  in  those 
prone  to  such  seizures,  and  the  hyperaesthetic  areas  may 
become  true  hysterogenic  zones.  These  hysterogenic 
zones  have  been  studied  with  great  care  by  the  French 
school,  but  we  cannot  enter  upon  the  subject  more  fully 
here,  and  leave  it  with  the  simple  statement  that  there  are 
some  hysterogenic  zones  which  are  not  hyperaesthetic. 

Hysterical  anaesthesia  is  still  more  frequent  than  the 
exaggerations  of  sensory  impressions.  The  anaesthesia 
may  not  only  include  every  form  of  ordinary  sensory  per- 
ception but  also  the  special  senses.  The  distribution  of 
this  anaesthesia  is  one  of  the  most  valuable  signs  of  hysteria. 
It  occurs  in  the  form  of  a  complete  hemianesthesia,  or  in 
the  form  of  a  regional  anaesthesia.  The  hemianesthesia  is 
limited  strictly  to  one-half  of  the  body,  but  in  this  half  it 
is  often  absolute,  and  the  completeness  of  the  anaesthesia  is 
the  very  feature  which  should  make  us  suspicious  of  its 
hysterical  nature.  If  a  girl  or  a  boy,  or  for  that  matter  any 
person  at  any  age,  present  absolute  anaesthesia  to  all  forms 
of  ordinary  sensation,  and  in  addition  exhibits  blindness  of 
one  side  or  a  typical  hemianopsia,  deafness,  loss  of  taste, 
and  of  smell  in  the  same  half  of  the  body,  such  a  person 
has  undoubtedly  hysteria.  In  this  country  hysterical 
hemianaesthesia  is  relatively  rare,  excepting  in  the  Russian 
and  French  elements  of  our  population,  but  in  these  it  can 
be  studied  easily  and  satisfactorily. 

If  an  entire  half  of  the  body  is  not  anaesthetic,  the  re- 
gional character  of  an  anaesthesia  is  sufficient  to  lead  one  to 
the  diagnosis  of  hysteria.  By  regional  anaesthesia  I  mean 
anaesthesia  of  a  well-marked  division  of  the  body,  say  of 
the  hand,  of  the  forearm,  of  the  leg,  the  hips,  or  of  circum- 
scribed areas  in  any  part  of  the  body  without  reference  to 
the  anatomical  distribution  of  the  sensory  nerves.  (Fig.  45.) 
As  this  regional  anaesthesia  is  frequently  associated  with  a 
regional  paralysis  the  hysterical  nature  of  both  becomes 
very  evident.  The  hysterical  nature  of  the  anaesthesia  is 
not  only  made  evident  by  its  peculiar  manner  of  develop. 


HYSTERIA. 


99 


ment,  but  also  by  the  manner  in  which  it  often  disappears. 
In  a  number  of  cases  in  which  I  have  studied  this  anaesthesia 
carefully  it  would  recede  inch  by  inch,  but  always  main- 
tained a  certain  level  in  the  arms  or  legs  without  any  ref- 
erence to  the  well-known  sensory  areas.  Hysterical  pa- 
tients do  not  present  anaesthesia  of  the  anterior  or  posterior 
surface  of  a  limb,  but  the  anaesthesia  generally  involves 
both  surfaces.  The  hysterical  nature  of  sensory  disturb- 
ances is  also  established  by  the  powerful  effect  of  hypnot- 


FiG.  45. — The  Three  Types  of  Distribution  of  Anaesthesia  in  Hysteria:  Hemianaes- 
thesia,  Segmental,  and  Disseminated.     Z,  Hysterogenic  Zones.     (Dana.) 


ism,  and  of  mere  suggestion,  which  is  often  sufficient  at 
least  temporarily  to  dislodge  a  complete  anaesthesia.  The 
anaesthesia  may  furthermore  be  transferred  from  one  limb 
to  another,  not  merely  by  the  action  of  magnets,  or  by  the 
action  of  metals  as  was  formerly  supposed,  but,  I  am  con- 
vinced, by  any  form  of  suggestion  powerful  enough  to  pro- 
duce a  strong  psychic  impression.  It  is  on  the  theory  of 
suggestion  that  we  must  explain  the  miracles  ascribed  to 
magneto-therapy  or  metallo-therapy,  that  played  such  an 
important  role  about  twenty-five  years  ago  in  France. 

Of  the  special  senses,  vision  is  most  frequently  subject 
to  hysterical  disorder.  In  some  there  is  true  photophobia, 
in  others  a  diminution  of  visual  perception  is  more  common, 


IOO 


THE  NERVOUS  DISEASES    OF   CHILDREN. 


and  the  patient  may  indeed  be  unaware  of  the  existence  of 
such  diminished  visual  sensation,  which  is  the  best  proof 
that  it  is  not  intentional  or  simulated.  The  retina  may  be 
entirely  insensitive  to  light,  there  may  be  distinct  limita- 
tion of  the  visual  field,  or  there  may  be  a  complete  loss 
of  every  form  of  visual  perception  in  one  eye.  (Fig.  44.) 
Bilateral  loss  of  sight  is  said  to  occur  in  hysterical  patients, 
but  as  a  rule  it  is  simply  transitory.  I  have  not  had  op- 
portunity to  see  this  special  visual  defect  in  children. 

Visceral  hysteria  deserves  a  passing  notice  ;  the  paral- 
ysis of  the  bladder  and  increased  peristaltic  action  of  the 


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Fig.  46. — Hysterical  Loss  of  Color  Sense  and  Limitation  of  Visual  Field.  Color 
Sense  Absent  in  Left  Eye  and  Field  Contracted  ;  in  Right  Eye  Field  less  Con- 
tracted ;  Order  of  Appreciation  of  Colors  from  Without  In  was  Yellow,  Violet,  Blue, 
Red,  Green.     (After  Peck,  from  Dana.) 


stomach  and  bowels  have  been  mentioned  in  connection 
with  the  motor  disturbances  of  hysteria,  but  there  are  two 
other  forms  of  hysterical  manifestations  that  are  worthy  of 
further  mention.  The  one  form  is  hysterical  anorexia. 
Patients  in  this  condition  absolutely  refuse  food,  and  if  they 
take  it,  vomit  it  at  once.  In  some  instances  this  is  accom- 
plished without  effort  on  the  part  of  the  patient  and  seems 
entirely  unintentional.  In  other  instances,  again,  the  pa- 
tient deliberately  sets  to  work  to  eject  the  contents  of  the 
stomach,  and  does  not  give  up  the  effort  until  she  has  suc- 
ceeded.   In  my  service  at  the  Montefiore  Home  I  frequent 


HYSTERIA.  10 1 

ly  had  occasion  to  witness  the  antics  of  an  hysterical  girl, 
aged  fifteen,  who  immediately  upon  taking  food  would  go 
into  a  corner  of  the  room,  begin  violent  gagging  movements, 
and  would  succeed  within  a  period  of  three  or  four  minutes 
in  ejecting  the  entire  contents  of  the  stomach.  In  this  girl, 
who  was  not  possessed  of  any  insane  delusion  regarding 
the  character  of  the  food,  the  act  seemed  purely  volitional, 
and  it  was  a  surprising  fact  that  like  many  other  hysterical 
patients  with  anorexia,  she  was  not  so  much  reduced  in  her 
physical  condition  as  a  person  would  have  been  who  had 
persistently  starved  herself.  She  evidently  obtained  food 
and  retained  it ;  but  how  she  got  it  and  when  she  took  it 
it  was  difficult  to  determine,  since  she  was  watched  care- 
fully night  and  day.  She  was  treated  by  rather  heroic 
measures  in  the  form  of  forced  feeding  and  hydro-thera- 
peutic measures ;  she  was  allowed  to  leave  the  institution 
after  she  had  shown  her  willingness  to  take  food  and  re- 
tain it. 

Distention  of  the  stomach  and  bowels,  representing  a 
total  paralysis  of  the  muscular  walls  of  the  stomach  and  in- 
testines, occurs  frequently  enough,  and  more  particularly  in 
children.  The  stomach  is  apt  to  be  distended  to  an  enor- 
mous degree,  and  the  bowels  dilate  in  such  a  way  as  to  give 
the  appearance  of  the  abdomen  in  a  case  of  large-sized 
tumor.  I  have  in  mind  the  cases  of  twin  brothers  who  pre- 
sented the  following  peculiar  condition  :  The  one  brother, 
at  the  age  of  fourteen,  was  true  to  the  neurotic  stock  from 
which  he  descended  by  suddenly  developing  a  tremendous 
meteorismus,  which  increased  from  day  to  day  until  the 
abdomen  was  so  distended  that  the  skin  seemed  ready  to 
burst.  There  was  some  anorexia  and  constipation.  The 
constipation  was  occasionally  relieved,  with  the  result  of 
temporarily  diminishing  the  distention  of  the  bowels,  but 
in  a  very  few  hours  the  intestines  would  resume  their  for- 
mer state.  Every  possible  measure  was  resorted  to,  to  re- 
move this  distention,  but  it  persisted  stubbornly  for  some 
weeks,  when  finally,  after  the  introduction  of  large  enemata 
of  an  infusion  of  valerian,  the  distended  abdomen  suddenly 
collapsed.  A  few  days  later  the  twin  brother  of  this  patient 
began  the  same  performance,  and  went  through  exactly  the 


102  THE   NERVOUS  DISEASES    OF   CHILDREN. 

same  experiences.  The  abdomen  became  distended  to  almost 
the  same  degree  as  in  the  brother,  and  after  it  had  lasted 
about  the  same  length  of  time  his  abdomen  also  collapsed  in 
very  much  the  same  way.  These  boys  not  only  came  from 
very  neurotic  stock,  but  had  been  very  much  pampered  in 
their  early  training,  which  fertilized  the  soil  that  had  been 
prepared  for  the  development  of  every  form  of  hysteria. 

Diagnosis. — It  would  be  impossible  to  state  the  points 
of  differential  diagnosis  between  hysteria  and  other  affec- 
tions without  repeating  everything  that  has  been  said  in 
the  preceding  paragraphs.  It  is  worth  while  stating  once 
more  that  the  diagnosis  of  hysteria  should  be  made  only  in 
case  an  organic  affection  can  be  positively  excluded,  and  if 
the  well-recognized  symptoms  of  hysteria,  particularly  hys- 
terical seizures  and  hysterical  sensory  changes  are  present. 
Let  the  student  also  bear  in  mind  that  hysteria  and  hypo- 
chondriasis are  not  one  and  the  same  disease,  though  one 
is  often  mistaken  for  the  other.  If  a  boy  is  fearful  of  a  dis- 
ease with  which  he  supposes  he  is  afflicted,  we  have  no 
hesitation  in  saying  that  he  is  a  hypochondriac ;  but  let  a 
girl  exhibit  exactly  the  same  symptom,  and  she  is  at  once 
put  down  as  an  hysterical  subject.  To  distinguish  between 
the  two  conditions  is  not  always  an  easy  matter,  but  in 
hypochondriasis  the  patient  is  generally  possessed  of  a  few 
notions  regarding  his  own  bodily  condition,  and  these  make 
such  a  deep  impression  upon  him  that  his  entire  ego  is  af- 
fected by  it.  His  supposed  affliction  is  constantly  before 
his  mind,  and  dominates  his  entire  being.  In  cases  of  hys- 
teria there  is  no  such  introspection  as  in  hypochondriasis. 
The  bodily  ailments  or  bodily  peculiarities  are  far  more 
numerous,  but  they  are  subject  to  greater  changes,  disap- 
pear for  a  time  and  then  return  again,  and  are  not  pushed 
to  the  fore  quite  as  much  as  in  cases  of  hypochondriasis. 
In  the  latter,  moreover,  the  depression  is,  as  a  rule,  greater 
than  in  hysteria,  and  in  hysteria  there  are  other  symptoms 
which  prove  the  presence  of  an  hysterical  affection.  The 
difficulties  of  a  differential  diagnosis  are  still  further  in- 
creased by  the  fact  that  a  mixture  of  the  two  conditions 
(hysterical  hypochondriasis)  is  not  uncommon,  and  it  must 
be  left  to  the  physician  to  determine,  by  a  close  analysis 


HYSTERIA.  103 


of  the  symptoms,  whether  there  is  more  hysteria  or  more 
hypochondriasis  in  the  symptoms  which  the  patient  pre- 
sents. 

Pathology. — In  discussing  the  pathology  of  hysteria 
there  is  much  room  for  theorizing,  but  there  are  very  few 
facts  to  guide  us.     That  it  is  a  functional  disease  is  con- 
ceded on  all  sides;  but  there  is  no  other  disease  in  which 
the  loss  of  function  may  be  so  absolute  as  in  hysteria.     By 
some  mechanism  or  by  some  influence  which  we  cannot 
yet  understand,  an  entire  hemisphere  is  temporarily  invali- 
dated, or  else  we  could  not  explain  the  typical  and  com- 
plete hemianesthesia   so   common   in   hysteria.      Meynert 
endeavored  to  give  an  anatomical  explanation,  but  failed. 
How  this  loss  of  function  of  one  hemisphere  is  effected,  and 
whether  it  is  similar  or  not  to  the  occurrences  that  take 
place  under  hypnotic  influence,  we  are  not  prepared  to  say. 
The  transfer  of  sensory  disturbances  from  one-half  of  the 
body  to  the  other,  would  go  to  show  that  the  two  halves 
of  the  brain  are  evidently  in  sympathy  with  one  another 
(to  put  it  broadly)  in  this  disease,  and  that  they  can  be  al- 
ternately affected.     The  highest  centres  are  evidently  im- 
paired in  hysteria,  cortical  inhibition  is  removed,  and  the 
lower  centres  have  full  sway*     It  is  quite  in  keeping  with 
this  view  that  even  the  reflexes  are  frequently  exaggerated 
in  hysteria  as  they  are  in  organic  disease  in  which  the 
changes  in  the  motor  fibres  of  the  pyramidal  tract  interfere 
with"  the  proper  transmission  of  cortical  influences.      We 
cannot  properly  speak  of  the  morbid  anatomy  of  hysteria, 
for  the  entire  conception  of  the  disease  would  have  to  be 
altered  if  we  could  suppose  the   existence  of  such.     Some 
post-mortem  findings  have  been  reported,  but  they  were 
evidently  accidental  complications. 

Duration  and  Course.— Once  hysterical  always  hys- 
terical, would  seem  to  be  the  general  opinion  with  regard 
to  these  subjects,  but  this  is  not  quite  accurate.  The  mani- 
festations of  this  disease  often  disappear  for  years,  and  it  is 

*  Breuer  and  Freud  have  attempted  a  psychological  explanation  of  hysterical  phe- 
nomena and  have  ascribed  these  to  a  "  hypnoid  "  condition  in  which  there  is  a  division 
of  consciousness,  and  a  limitation  of  the  power  of  association.  Freud  has  directed  at- 
tention to  the  diminution  in  the  strength  of  concepts  in  hysterical  subjects,  and  to  the 
ready  change  from  psychic  to  physical  innervation. 


104  THE  NERVOUS  DISEASES   OF   CHILDREN. 

one  of  the  commonest  experiences  to  record  the  disappear, 
ance  of  an  hysterical  paralysis  or  of  an  hysterical  anaes- 
thesia. The  tendency  to  relapses  is  extremely  great,  and 
symptoms  that  have  disappeared  for  years  may  occur  un- 
der any  emotional  excitement. 

Treatment. — The  treatment  of  hysteria  must  be  con- 
sidered with  reference  to  the  hysterial  predisposition  and 
with  reference  to  the  special  hysterical  symptoms.  The 
hysterical  predisposition  is  unfortunately  either  inherited 
from  a  neurotic  ancestry  or  it  has  been  specially  fostered 
by  an  hysterical  environment.  In  hysteria,  as  in  other  men- 
tal affections,  I  have  often  doubted  whether  heredity  is  the 
marked  factor  that  it  is  generally  supposed  to  be,  or,  if  we 
grant  the  hereditary  tendency,  whether  the.  hysterical  man- 
ifestations would  be  as  frequent  as  they  are  if  a  serious  at- 
tempt were  made  to  change  the  surroundings  of  such  chil- 
dren. In  the  majority  of  cases  the  early  training  has  been 
defective,  and  an  example  constantly  before  the  child  of  an 
hysterical  mother  or  other  hysterical  relatives  is  sufficient 
to  engender  the  disease  in  its  fullest  form.  The  first  and 
most  important  principle  of  treatment,  therefore,  is  the  ab- 
solute separation  of  the  child  from  the  family.  It  is  a  great 
pity  that  this  is  so  rarely  urged  by  the  physician,  and  still 
more  rarely  permitted  by  the  parents.  Only  the  more  in- 
telligent parents  can  be  made  to  understand  that  an  utter 
stranger,  if  properly  qualified,  may  train  a  child  far  better 
than  its  own  mother  can.  At  all  events  there  is  nothing  in 
the  treatment  of  hysterical  children  as  important  as  plac- 
ing them  under  the  influence  of  a  sober-minded,  intelligent 
nurse  or  teacher  who  will  devote  herself  or  himself  to  study- 
ing the  peculiarities  of  the  child,  and  who  will  make  a  seri- 
ous attempt  to  foster  the  good  qualities  and  to  counteract 
the  vices.  If  this  course  that  has  been  suggested  is  per- 
sisted in,  it  should  be  followed  not  for  weeks  or  months  but 
for  years,  and  I  am  confident  that  if  this  is  done  during  the 
formative  period  of  a  child's  mind  and  character,  an  excel- 
lent result  will  follow  in  many  instances.  I  base  this  upon 
my  own  experience,  and  have  often  stated  to  parents  that 
if  they  will  not  allow  me  to  pursue  such  a  plan  of  treatment 
I  prefer  not  to  attempt  any. 


HYSTERIA.  105 

The  Weir-Mitchell  treatment,  which  is  so  effective  in 
many  cases  of  adult  hysteria,  is  also  of  the  greatest  value  in 
the  hysteria  of  children  ;  but  I  consider  it  to  be  one  of  its 
special  advantages  that  it  presents  the  first  and  easiest  op- 
portunity for  the  separation  of  the  child  from  its  immediate 
surroundings.  Select  a  nurse  carefully,  place  the  child  en- 
tirely in  her  charge,  and  many  a  parent  will  soon  be  con- 
vinced of  the  good  that  comes  from  such  separation.  In 
addition  to  the  moral  influences  which  may  be  exerted  in 
this  way  the  physical  health  of  the  child  can  be  looked 
after.  Bad  habits  of  diet  and  of  general  hygiene  can  be 
corrected,  and  a  child  whose  general  physical  condition 
has  been  far  below  par  up  to  the  time  of  its  entering  upon 
the  rest  cure,  may  be  turned  into  a  strong  and  vigorous  be- 
ing. Drugs,  I  am  very  certain,  have  but  little  influence 
over  such  conditions.  The  usual  hysterical  remedies,  such 
as  asafcetida,  valerian,  and  the  like,  may  be  employed,  but 
they  will  accomplish  little  which  cannot  be  accomplished 
by  other  means  as  well.  If  the  effect  of  a  drug  depends 
upon  its  disagreeable  taste,  suggestions  of  a  different  char- 
acter will  prove  quite  as  efficient ;  and  above  all  the  phy- 
sician should  avoid  the  possible  risk  of  engendering  the 
idea  in  a  growing  child  that  there  is  a  special  drug  which 
it  may  resort  to  for  any  annoying  or  painful  sensation. 
There  is  nothing  more  disgusting  than  the  habit  so  freely 
practised  by  many  physicians  of  giving  valerian  or  asafce- 
tida or  morphine  to  children  or  adults,  whenever  they  pre- 
sent symptoms  which  smack  of  hysteria,  or  which  cannot 
be  interpreted  otherwise  by  the  attending  medical  adviser. 

The  special  symptoms  of  hysteria  call  for  distinct  forms 
of  treatment,  but  whatever  these  special  symptoms  may  be,  I 
always  consider  it  wise  to  bear  the  general  hysterical  condi- 
tions in  mind,  and  to  employ,  in  addition  to  the  special  treat- 
ment, those  general  measures  which  have  an  excellent  invigo- 
rating effect  upon  the  nervous  system.  I  refer  more  particu- 
larly to  the  proper  use  of  hydrotherapeutic  measures.  The 
treatment  of  hysterical  aphonia  was  referred  to  above.  Sim- 
ple faradism  is  all  that  is  needed  in  most  instances,  and  if  this 
is  not  sufficient,  regular  vocal  exercise  in  the  hands  of  an  in- 
telligent teacher  or  nurse,  will  bring  about  the  desired  effect. 


106  THE  NERVOUS  DISEASES   OF   CHILDREN. 

I  have  never  found  it  necessary  to  apply  the  faradic  current 
to  the  inside  of  a  throat,  but  of  course  would  not  hesitate 
to  do  so  in  case  the  external  application  did  not  prove  suf- 
ficient. Blistering  or  the  application  of  the  static  current  acts 
as  a  powerful  form  of  local  suggestion.  In  hysterical  paral- 
ysis of  the  limbs,  the  use  of  a  strong  faradic  current  is  gener- 
ally the  most  effectual  remedy.  The  current  applied  know- 
ingly, not  mercilessly,  will  gradually  induce  the  patient  to 
attempt  similar  contractions ;  and  if  to  the  use  of  the  faradic 
current  be  added  the  encouraging  words  of  the  nurse  or 
physician,  who  should  not,  however,  accuse  the  child  of 
simulation,  and  if  other  measures,  such  as  massage,  be  em- 
ployed, the  hysterical  paralysis  will  disappear  in  the  course 
of  time.  But  many  of  the  cases  are  extremely  stubborn 
and  may  require  more  vigorous  measures,  or  more  power- 
ful suggestion,  before  they  yield  to  treatment.  It  is  of 
some  importance  at  times  to  prove  to  the  child  that  it  can 
use  its  limbs  very  much  better  than  it  supposed.  Place  it 
in  the  middle  of  a  room  quite  by  itself,  make  it  stand  or 
crawl  or  walk,  and  if  once  shown  that  it  can  do  so,  the  para- 
lytic symptoms  may  rapidly  disappear.  But  the  treatment 
of  hysterical  patients  of  all  ages  and  of  all  classes  demands 
unusual  tact  and  patience  on  the  part  of  everyone  con- 
cerned in  the  treatment  of  the  case.  • 

The  sensory  disturbances  of  hysteria  are  best  influenced 
by  the  use  of  the  cold  douche  or  of  the  faradic  current, 
particularly  by  the  employment  of  the  faradic  brush.  If 
such  measures  as  I  have  suggested  are  not  sufficient  to  re- 
move an  hysterical  paralysis  or  an  hysterical  anaesthesia 
the  effect  of  suggestion,  hypnotic  or  otherwise,  may  be 
attempted. 

The  visceral  disturbances  common  in  hysteria  also  de- 
mand special  treatment.  Some  of  the  measures  to  be  em- 
ployed were  referred  to  in  connection  with  the  hysterical 
twins  mentioned  above.  In  cases  of  hysterical  anorexia 
patient  efforts  should  be  made  to  induce  the  patient  to  take 
small  quantities  of  food,  and  if  these  are  not  retained  forced 
feeding  must  be  resorted  to  ;  but  never,  if  you  can  avoid  it, 
resort  to  rectal  feeding,  for  the  patient  who  has  discovered 
that  she  can  be  fed  in  that  way  will  continue  to  refuse  food 


HYSTERIA.  I07 

very  much  longer  than  she  would  otherwise.  Lastly,  the 
treatment  of  hysterical  attacks  may  be  managed  in  very 
much  the  same  way  as  other  hysterical  manifestations  are 
treated.  First  of  all,  the  patient  should  receive  the  general 
anti-hysterical  treatment,  and  should  be  given  some  drug 
which  will  act  as  a  powerful  irritant  or  a  powerful  form  of 
suggestion  when  the  attack  is  imminent.  I  have  found 
nothing  better  than  a  sudden  douche  of  cold  water,  or  cold 
flagellations  on  the  head  and  chest,  or  the  inhalation  of  ni- 
trite of  amyl.  The  latter  has  this  to  recommend  it,  that  it 
may  be  used  with  all  the  more  assurance  in  those  cases  in 
which  the  suspicion  of  epilepsy  cannot  be  altogether  ex- 
cluded. Lavender  and  ammonia  are  also  efficient  in  some 
cases  in  which  the  hysterical  attack  can  be  inhibited  by  any 
such  simple  measure.  If  an  hysterical  attack  continue  for 
a  prolonged  period  of  time  it  may  be  brought  to  an  end 
by  the  brisk  use  of  cold  douches,  by  the  application  of  a 
strong  faradic  current,  or  by  pressure  over  the  ovaries, 
after  the  age  of  puberty,  if  these  organs  be  oversensitive. 


CHAPTER  V. 

CHOREA. 

Among  the  neuroses  of  childhood  none  is  worthier  of 
careful  study  than  chorea.  The  name  is  made  by  some  to 
include  a  number  of  varying  conditions,  but  its  use  should 
be  restricted  to  designate  a  functional  disease  characterized 
by  irregular,  involuntary  twitchings  of  some  or  all  of  the 
muscles  of  the  body.  These  movements  cease,  as  a  rule, 
during  sleep. 

Synonyms. — This  neurosis  is  also  known  as  St.  Vitus's  Dance  and  the 
Chorea  of  Sydenham.  Both  these  names  deserve  to  be  used,  since  the  former 
implies  its  historical  origin,  and  in  the  latter  is  preserved  the  name  of  the 
famous  English  physician  who  first  described  its  most  characteristic  symp- 
toms. Scelotyrbe  and  Melancholia  saltans,  are  terms  occasionally  used, 
though  almost  obsolete.  In  German  text-books  the  disease  is  called  Chorea 
Minor,  in  contradistinction  to  Chorea  Major,  a  neurosis  of  a  purely  hysterical 
character.* 

ETIOLOGY. — Chorea  is  distinctly  a  neurosis  of  childhood 
and  early  adolescence.  The  vast  majority  of  cases  begin  in 
very  early  youth,  though  Sinkler,  many  years  ago,  reported 
two  cases  in  persons  over  eighty  years  of  age.  Careful  sta- 
tistics have  been  gathered  with  reference  to  this  disease, 
the  most  elaborate  being  those  reported  by  Dr.  Stephen 
Mackenzie,  in  1887,  for  the  British  Medical  Association  Col- 
lective Investigation  Committee.  Of  four  hundred  and 
thirty-nine  cases  reported  by  this  committee  thirty-four  per 
cent,  occurred  between  the  ages  of  five  and  ten  years,  forty- 

*The  name  chorea,  Greek  x°Pe'a,  t;an  be  traced  back  to  the  dancing  mania  of  the 
middle  ages.  During  a  severe  outbreak  of  this  psychic  disturbance  in  Strasburg,  in 
the  early  part  of  the  fifteenth  century,  the  chief  magistrate  of  that  city  ordered  those 
affected  with  this  dancing  mania  to  repair  to  the  chapel  of  St.  Vitus,  in  Zabern,  a  small 
village  not  far  from  Strasburg.  The  name,  St.  Vitus's  Dance,  is  the  only  point  of  affin- 
ity between  the  dancing  mania  of  old  and  the  typical  chorea  of  the  present  day. 


CHOREA.  I09 

three  per  cent,  between  the  ages  of  ten  and  fifteen  years, 
and  sixteen  per  cent,  between  the  ages  of  fifteen  and  twenty 
years.  The  largest  number  of  attacks  were  found  to  occur 
in  the  thirteenth  year  of  life.  Some  cases,  however,  oc- 
curred very  much  earlier  than  this  average  percentage 
would  indicate.  I  have  seen  several  cases  of  genuine  chorea 
in  children  under  one  year  of  age,  and  many  more  in 
children  up  to  the  age  of  three  years.  That  chorea  is 
also  occasionally  congenital  must  be  admitted  :  Sinkler  re- 
fers to  a  case  of  this  description  due  to  fright  of  the  mother 
during  pregnancy.  That  there  is  hereditary  predisposition 
to  chorea  is  also  evident,  for  the  disease  is  developed  more 
readily  in  children  of  choreic  mothers  and  also  in  those 
whose  parents  are  afflicted  with  epilepsy  or  migraine. 

All  authors  are  agreed  as  to  the  greater  liability  of  the 
female  sex.  Within  a  period  of  three  years  I  had  seen  184 
cases  of  chorea,  in  which  136  were  females  and  48  males. 
Sinkler  among  328  cases  reports  232  females  and  96  males. 
Gowers,  who  has  tabulated  the  largest  number  of  cases,  finds 
only  365  boys  among  1,000  cases. 

Dr.  Weir  Mitchell  has  studied  the  relation  of  races  to 
the  development  of  chorea,  and  claims  that  the  negro  race 
is  almost  exempt.  I  have  no  means  of  saying  whether  this 
is  approximately  true,  but  from  my  own  experience  I  can 
assert  that  it  is  by  no  means  rare  among  that  race,  but  that 
among  them  it  is  if  anything  more  frequent  in  boys  than 
in  girls.  The  disease  is  very  common  in  Hebrews,  as  are 
many  other  neuroses. 

Climate  seems  to  exercise  but  very  little  influence  upon 
the  development  of  the  disease  proper,  or  upon  the  causa- 
tion of  the  individual  attack  ;  it  occurs  quite  as  freely  in 
cold  countries  as  in  warm,  in  northern  as  in  southern  lati- 
tudes, but  there  is  an  undoubted  seasonal  influence.  The 
great  majority  of  attacks  occur  in  the  spring.  Drs.  Mitch- 
ell and  Lewis  have  made  an  elaborate  research  on  this  point, 
and  claim  that  it  is  not  so  much  variation  in  temperature 
or  in  humidity  that  causes  the  attacks,  but  that  there  is  a 
decided  correspondence  between  the  number  of  attacks  of 
chorea  and  the  number  of  rainy  and  cloudy  days ;  and  then 
again  between  the  attacks  of  chorea  and  the  number  of  storm- 


IIO  THE   NERVOUS  DISEASES    OF   CHILDREN. 

centres  that  pass  over  Philadelphia.  Too  much  reliance 
should  not  be  placed  upon  these  statements  until  confirmed 
by  similar  researches  in  other  cities.  Putnam  could  not 
trace  the  same  influences  in  Boston,  and  Gowers  cannot 
accept  the  conclusions  of  Lewis  for  the  conditions  under 
which  chorea  occurs  in  England.  These  factors,  if  power- 
ful at  all,  are  of  more  importance  in  bringing  about  a  recur- 
rence of  attacks  that  in  giving  the  first  impetus  to  the  dis- 
ease. 

The  causes  which  lead  most  directly  to  the  development 
of  St.  Vitus's  Dance  are  (in  the  order  of  their  importance) 
fright,  various  acute  diseases,  such  as  articular  rheuma- 
tism, scarlatina,  and  cardiac  disease  which  so  often  ac- 
companies the  acute  affections  just  mentioned.  Gowers  is 
inclined  to  regard  fright  as  "  the  only  immediate  cause  that 
can  be  traced  with  any  frequency."  It  was  the  direct  cause 
of  chorea  in  56  of  my  184  cases.  As  a  rule,  the  first  symp- 
toms of  chorea  appear  within  a  few  days  of  the  fright ;  in 
some  cases  within  a  few  hours,  and  even  within  the  first 
hour.  I  had  under  observation  for  a  long  time  a  child  that 
lived  near  the  Brooklyn  Theatre  at  the  time  it  was  destroyed 
by  fire ;  the  child  was  startled  by  the  sight  of  the  flames, 
and  within  a  few  hours  began  to  twitch,  and  soon  developed 
a  severe  attack  of  chorea  which  lasted  for  months.  In  one 
case  the  sight  of  a  street  brawl,  in  another  the  sight  of  a  dead 
body,  was  directly  the  cause  of  the  choreic  attack..  In  chil- 
dren who  have  once  had  chorea  very  trivial  occurrences 
are  apt  to  bring  about  a  recurrence.  I  have  known  a  slight 
overstrain  at.  school,  the  unexpected  report  of  a  pistol,  a 
severe  thunderstorm,  or  a  scolding  by  a  parent  to  be  suf- 
ficient to  bring  on  an  attack. 

The  relation  of  acute  rheumatism  to  the  development  of 
chorea  has  always  been  a  matter  of  dispute.  Some  claim 
an  absolute  causal  relation  between  the  two,  others  insist 
that  it  is  nothing  more  than  a  coincidence  if  one  disease 
follows  upon  the  other.  Statistics  upon  this  point  are  not  so 
satisfactory  as  they  might  be,  for  cases  have  been  reported 
in  which  it  was  stated  that  acute  rheumatism  has  occurred, 
but  it  is  not  clearly  made  out  that  the  rheumatism  actually 
preceded  the  onset  of  the  chorea.     See  reported  the  occur- 


CHORE  A.  I  I  I 

rence  of  acute  rheumatism  in  about  one-fourth  of  his  cases, 
and  in  these  figures  he  is  supported  by  the  conclusions  of  the 
British  Medical  Investigation  Committee  and  by  Gowers.* 
It  is  a  curious  fact  the  rheumatism  does  not  seem  to  pre- 
cede chorea  nearly  so  frequently  in  earlier  years  as  it  does 
in  those  cases  which  occur  between  the  ages  of  ten  and  fif- 
teen years.  From  this  we  must  infer  that  accidental  coin- 
cidence plays  a  very  much  greater  role  than  many  are  will- 
ing to  concede.  This  must  unquestionably  be  the  case  with 
the  statistics  furnished  by  Dr.  Hamilton,  who  found  twenty 
per  cent,  of  all  school  children  choreic  or  affected  with  some 
similar  disorder.  I  could  find  a  satisfactory  history  of  the 
chorea  coming  on  after  rheumatism  in  only  20  of  184-cases.t 
Hirt,  in  his  recent  text-book  on  Nervous  Diseases,  thinks  that 
there  is  a  common  toxic  agent  which,  if  it  affects  the  cor- 
tex, will  produce  choreic  movements;  if  it  affects  the  joints 
chiefly,  will  give  rise  to  acute  rheumatism.  This  may  be  a 
rather  hasty  conclusion,  but  there  is  enough  truth  in  it  to 
say  that  the  development  of  chorea  is  more  probable  in  per- 
sons who  have  had  rheumatism  than  in  those  who  have  not. 
The  majority  of  recent  writers,  including  Herringham, 
Mackenzie,  Oppenheim,  Gowers,  Osier,  and  others  insist 
that  an  intimate  relation  exists  between  chorea  and  rheuma- 
tism ;  but  the  question  arises  whether  heart  disease,  which  is 
so  frequently  associated  with  rheumatism,  may  not  be  in  part 
responsible  for  the  development  of  chorea.  Statistics,  in 
order  to  be  satisfactory,  should  be  collected  very  much 
more  carefully  than  they  have  been  hitherto,  in  order  to 
prove  in  how  few,  or  in  how  many,  cases  of  chorea  the  first 
attack  has  been  preceded  by  rheumatism  or  cardiac  disease. 
We  can  readily  understand  that  fright  should  be  a  more 
powerful  agent  for  evil  in  cases  in  which  cardiac  disease 
has  preceded  the  existence  of  chorea.  I  have  found  satis- 
factory evidence  %  of  cardiac  disease  preceding  the  devel- 
opment of  chorea  in  only  20  of  184  cases.     The  bearing  that 

*  Starr  has  tabulated  2,476  cases  (by  various  authors);  in  662  (twenty-six  per  cent.) 
there  was  a  history  of  preceding  rheumatism  ;  in  502  there  was  cardiac  disease. 

t  Osier  states  that  there  was  a  history  of  rheumatism  in  15.8  per  cent,  of  cases  which 
he  had  examined  carefully. 

\  The  mere  existence  of  a  murmur  has  been  taken  by  many  as  evidence  of  cardiac 
disease  ;  in  the  fewest  reports  is  there  any  accurate  diagnosis  of  the  cardiac  condition. 


112  THE   NERVOUS  DISEASES    OF   CHILDREN: 

heart  disease  may  have  upon  the  pathology  of  chorea  we 
shall  have  occasion  to  refer  to  later  on. 

There  is  a  marked  tendency  at  the  present  time  to 
overrate  the  infectious  origin  of  chorea.  One  may  claim 
that  it  is  at  times  a  post  infectious  disorder.  To  claim 
more  would  be  unwarranted. 

There  has  been  much  talk  about  reflex  chorea,  as  about 
the  reflex  origin  of  many  other  neuroses,  but  he  who  sees 
with  only  half  an  eye  will  soon  convince  himself  that  these 
reflex  theories  are  but  a  poor  makeshift.  Of  all  the  cases 
of  chorea  that  I  have  seen,  I  have  found  but  very  few  that  I 
could  consider  due  to  any  peripheral  exciting  cause.  I  have 
convinced  myself  that  in  a  few  cases  the  presence  of  intes- 
tinal parasites  was  the  cause  of  a  transitory  chorea,  which 
disappeared  as  soon  as  the  parasites  were  removed,  but  I 
am  not  convinced  that  nasal  or  ocular  trouble,  of  which  so 
much  has  been  made  of  late,  ever  leads  to  true  chorea.  If 
these  troubles  prove  an  inconvenience  to  the  child,  some 
choreiform  habits  may  for  a  time  be  established,  but  in 
such  cases  the  cardinal  symptoms  of  St.  Vitus's  Dance  are 
wanting. 

There  is  a  curious  relation  between  epilepsy  and  chorea. 
Gowers  refers  to  epilepsy  developing  from  chorea,  and  I 
have  seen  a  case  in  a  woman  of  forty,  and  another  in  a  child 
of  ten  years,  in  which  severe  chorea  set  in  after  the  cessa- 
tion of  epileptic  attacks. 

Symptoms. — Involuntary  and  irregular  movements  of 
any  muscle  or  group  of  muscles  of  the  body  constitute  the 
chief  symptom  of  chorea.  The  muscles  of  the  hands  and 
fingers,  and  of  the  face  and  tongue  are  most  often  affected, 
but  the  leg  and  trunk  muscles  are  at  times  involved.  These 
movements  are  aggravated  by  volitional  effort  either  of  the 
muscles  affected  or  of  some  other  group  of  muscles.  Thus 
the  choreic  movements  of  the  hands  will  often  become  very 
much  more  intense  if  the  child  is  told  to  stand  absolutely 
still,  or  if  while  one  hand  is  being  examined  it  is  asked  to 
grasp  something  with  the  other  hand.  If  the  patient  at- 
tempt to  keep  the  affected  part  absolutely  quiet  he  may 
succeed  in  doing  so  for  a  few  seconds,  but  after  that  the 
movements  will  become  more  intense. 


CHOREA.  113 

The  choreic  movements  may  affect  only  one  extremity ; 
they  may  involve  one-half  of  the  body  (hemichorea),  or  they 
may  be  generalized.  In  184  cases,  35  were  cases  of  right 
hemichorea;  32  of  left  hemichorea;  and  in  117  cases  the 
choreic  movements  were  general. 

As  a  rule  the  choreic  movements  are  so  evident  that  no 
special  examination  is  needed.  As  the  child  enters  the  con- 
sulting-room the  most  superficial  inspection  is  sufficient  for 
a  diagnosis;  but  in  other  cases,  particularly  during  the  early 
stages  of  the  disease,  the  choreic  movements  are  discovered 
only  upon  careful  examination.  If  there  is  any  doubt  what- 
ever about  the  condition,  I  ask  the  child  to  place  its  hand 
quietly  upon  my  own,  or  between  my  two  hands ;  the  irreg- 
ular choreic  movements  will,  if  present,  be  easily  seen  or 
felt.  The  true  nature  of  the  trouble,  which  may  have  ap- 
peared to  be  nothing  more  than  "a  slight  nervousness,"  may 
thus  be  detected.  If  any  further  corroboration  is  needed,  an 
examination  of  the  tongue,  as  a  rule,  reveals  the  true  char- 
acter of  the  disease,  for  there  are  very  few  cases  of  chorea 
in  which  if  the  movements  of  the  extremities  are  ever  so 
slight,  the  tongue  does  not  exhibit  very  marked  choreic 
twitching.  These  tongue  movements  are  slow,  coarse, 
sometimes  rhythmical.  In  advanced  cases,  if  the  tongue 
is  protruded  the  mouth  is  opened  much  more  widely  than 
necessary,  the  eyelids  and  eyebrows  are  raised  in  the  same 
effort,  and  then  through  a  choreic  movement  of  the  mas- 
seters  the  tongue  may  be  caught  between  the  teeth.  These 
movements  combined  give  rise  to  what,  in  a  former  article, 
I  called  the  "  facies  "  of  chorea. 

The  movements  of  the  choreic  patient  are  not  only 
irregular  but  are  often  awkward  to  the  extreme.  This  is 
clearly  shown  in  the  attempt  to  open  or  button  the  cloth- 
ing, in  raising  a  glass  of  water  to  the  lips,  or  in  attempting 
to  hold  the  pen  in  writing.  This  awkwardness  often  in- 
duces great  irritability  on  the  part  of  the  child ;  but  how- 
ever annoying  the  movements  may  be,  it  is  only  in  a  very 
small  proportion  of  the  cases  that  they  lead  to  a  condition 
of  exhaustion.  A  few  years  ago  I  had  occasion  to  observe 
a  little  girl,  six  years  of  age,  the  child  of  healthy  and  intel- 
ligent  parents.     It   had   passed    successfully  through  one 


114  THE  NERVOUS  DISEASES    OF   CHILDREN. 

attack  of  chorea ,  and  in  the  second  attack,  coming  on  after 
a  fright,  the  movements  were  extreme,  and  sleep  was  so 
poor  that  within  a  few  weeks  the  child  died  from  exhaus- 
tion. A  weakened  but  not  diseased  heart  unquestionably 
assisted  in  bringing  about  this  early  fatal  termination. 
It  is  a  fortunate  circumstance  that  in  almost  all  these  cases 
the  movements  cease  during  sleep,  and  that  the  child  is 
thus  able  to  recover  partially  from  the  exhaustion  caused 
by  the  movements  during  the  day. 

Some  weakness  of  the  muscles  is  frequently  associated 
with  choreic  movements.  The  term  paralytic  chorea  has 
been  proposed  for  those  cases  in  which  there  is  marked 
paralysis,  but  as  there  is  more  or  less  weakness  in  the 
majority  of  the  cases,  and  often  more  awkwardness  than 
weakness,  there  does  not  seem  to  me  to  be  sufficient  excuse 
for  the  introduction  of  this  term. 

Speech  is  frequently  involved.  This  is  in  the  nature  of 
a  dysarthria  rather  than  an  aphasia,  the  choreic  movements 
of  the  tongue  and  laryngeal  muscles  making  speech  dif- 
ficult and  often  unintelligible.  In  some  cases  there  is  a 
little  awkwardness  of  articulation,  in  others  hasty  articu- 
lation leading  to  the  repetition  of  words,  and  in  some  a 
peculiar  condition  of  speech  which  is  in  part  due  to  diffi- 
culties of  articulation,  and  in  part  to  choreic  movements 
of  the  respiratory  muscles  necessitating  rapid  breathing. 
Deglutition  may  be  difficult,  the  tongue  is  frequently  bitten, 
and  from  the  awkwardness  in  the  use  of  the  knife  and  fork, 
and  in  passing  food  to  the  mouth,  the  little  patient  is  much 
annoyed  and  is  an  ungainly  sight  for  others  while  at  his 
meals. 

Laryngeal  chorea,  pure  and  simple,  occurs  somewhat 
rarely,  and  consists  of  choreiform  movements  of  the  muscles 
controlling  the  vocal  cord.  The  result  is  a  peculiar  ex- 
piratory noise  like  a  bark,  which  is  repeated  at  short  in- 
tervals. These  cases  are  often  mistaken  for  cases  of  hys- 
terical bark ;  but  the  general  restlessness,  the  age  of  the 
patient,  the  choreic  movements  of  the  tongue  and  fingers, 
should  leave  little  doubt  regarding  the  diagnosis.  I  re- 
member a  little  girl,  aged  ten,  who  began  to  bark  after  a 
sudden  fright ;  her  case  had  been  diagnosticated  as  hysteria 


CHOREA.  l  j  - 

by  several  eminent  physicians,  but  there  was  no  element  of 
true  hysteria  in  the  case.  She  recovered  promptly  under 
the  usual  rest  treatment.  There  is  little  doubt  in  my  own 
mind  that  this  represents  the  rarest  form  of  chorea. 

The  electrical  reactions  are  sometimes  slightly  altered 
in  cases  of  chorea.  Rosenthal,  Benedict,  and  others  have 
found  an  increased  response  to  the  faradic  and  galvanic 
currents  on  the  part  of  the  muscles  and  nerves  of  the  af- 
fected side.  Some  have  even  asserted  that  the  reaction  of 
degeneration  with  qualitative  galvanic  changes  occurs  in 
some  instances,  but  I  am  inclined  to  doubt  the  correctness 
of  this  statement.  I  have  never  found  a  similar  condition 
although  I  have  frequently  examined  patients  with  this  end 
in  view.  If  such  electrical  changes  were  present  I  should 
suspect  the  presence  of  multiple  neuritis,  as  this  has  been 
known  to  occur  together  with  chorea  ;  just  as  I  might  sus- 
pect this  same  complication  in  cases  of  marked  sensory  dis- 
turbances, for  the  rule  is  that  in  uncomplicated  chorea  sen- 
sation remains  undisturbed. 

Mental  disturbance  has  been  frequently  referred  to  by 
many  writers  as  a  complication  of  chorea.  It  is  surely 
not  a  very  frequent  occurrence,  except  that  in  the  cases 
of  chronic  chorea  (probably  a  different  disease)  the  ten- 
dency to  dementia  is  very  marked.  The  impression  I 
recorded  a  few  years  ago  seems  to  me  to  represent  the 
truth  of  the  matter :  "  The  mental  calibre  of  children  who 
develop  chorea  is  rather  above  than  below  par.  Children 
who  by  means  of  a  better  mental  development  stand  head 
of  the  class,  who  work  for  prizes  and  earn  them,  children 
who  are  under  constant  mental  strain,  and  about  whom 
parents  and  teachers  make  much  ado,  are  the  ones  most  apt 
to  be  attacked  by  chorea."  In  some  instances  a  violent 
mania  is  developed  early  {chorea  insaniens),  but  it  is  much 
rarer  to  find  this  sequence  of  events  than  to  observe  cases 
of  acute  mania,  particularly  among  young  girls,  in  whom 
the  movements  of  the  extremities  and  of  the  tongue  are 
typically  choreic.  Irritability  of  temper  is  perhaps  the 
most  frequent  mental  condition  associated  with  chorea ;  but 
this  is  natural  enough  if  we  consider  the  very  annoying 
movements  and   the  difficulty  the   child  sometimes  expe- 


Il6  THE  NERVOUS  DISEASES   OF   CHILDREN. 

riences  in  making  itself  understood.  At  times,  instead  of  a 
condition  of  mania,  a  condition  of  apathy  and  depression  is 
present  in  patients  afflicted  with  chorea  ;  but  I  am  inclined 
to  think  that  this  is  only  true  of  patients  who  inherit  a  pre- 
disposition to  mental  disease. 

The  temperature  has  been  studied  in  chorea.  In  mild 
cases  it  is  normal  throughout  the  entire  course  of  the 
disease.  In  severe  cases  it  may  be  raised  a  degree  or  two, 
but  any  greater  elevation  is  undoubtedly  due  to  some  other 
condition. 

Complications. — By  far  the  most  frequent  complica- 
tions are  rheumatism  and  heart  disease.  Rheumatism,  if 
present,  is  discovered  easily  enough  both  by  the  fever  and 
by  the  painful  swellings  ;  but  it  is  well  to  remember  that  the 
acute  rheumatism  of  children  is  often  a  very  much  vaguer 
disorder  than  the  acute  rheumatism  of  the  adult.  If  pain 
is  much  complained  of  in  any  case  of  chorea  the  joints 
should  be  examined  carefully.  Heart  disease  is  the  com- 
plication most  to  be  feared.  The  heart  should  therefore 
be  examined  frequently  and  carefully.  Mitral  regurgita- 
tion is  by  far  the  most  frequent  form  of  cardiac  disturb- 
ance. In  the  statistics  of  the  British  Investigation  Com- 
mittee there  were  116  cases  of  mitral  disease  and  only  6 
of  aortic  disease.  Gowers  found  but  two  instances  of  aortic 
regurgitation  among  252  cases  of  chorea.  Sinkler  found 
cardiac  murmur  in  82  of  279  cases,  but  he  does  not  decide 
how  many  were  due  to  organic  cardiac  disease.  It  may 
often  be  difficult  to  determine  this  question,  but  if  a  patient 
whose  heart  was  normal  develops  a  murmur  while  under 
observation  the  probability  of  organic  lesion  is  very  great ; 
yet  since  anaemia  is  very  frequent  in  cases  of  chorea,  we 
must  allow,  in  judging  cardiac  conditions,  for  the  possibility 
of  haemic  murmurs  and  slight  dilatation  of  the  heart. 
Brown  and  J.  K.  Mitchell  have  described  patients  covered 
with  subcutaneous  nodules.  These  have  a  more  direct  re- 
lation to  the  rheumatic  fever  than  to  the  chorea.* 

An  excess  of  urea  and  of  phosphates  has  been  found  in 
the    urine    of  choreic  patients.     It  is  questionable  at  best 

*  Osier  is  of  the  same  opinion  ;  he   considers  this  condition  a  great  rarity  in  this 
country. 


CHOREA.  I  I  7 

whether  they  are.  not  in  some  way  the  result,  of  the  inces- 
sant restless  movements.  Convulsive  attacks  are  referred 
to  as  a  complication  by  several  authors.  These  are  not  of 
a  typical  epileptic  character,  but  appear  to  be  half-choreic 
and  half-spasmodic  movements.  Mitchell  and  Burr  have 
recently  reported  a  case  of  this  sort.  The  cases  in  which 
epilepsy  is  associated  with  chorea  are  more  than  likely  cases 
of  organic  brain  lesion,  in  which  both  the  hemichorea  and 
the  epilepsy  are  symptoms  of  one  and  the  same  process  in 
the  cortex. 

DURATION. — It  is  difficult  to  give  any  accurate  informa- 
tion with  regard  to  the  duration  of  chorea,  as  the  disease 
can  hardly  be  said  to  be  ended  if  upon  the  slightest  prov- 
ocation another  attack  sets  in.  A  single  attack  may  last 
from  a  few  weeks  to  many  months.  The  average  duration 
is  generally  considered  to  be  about  ten  weeks.  In  my  own 
cases  the  duration  of  attacks  varied  between  four  and  twelve 
weeks.  Two  and  three  attacks  are  much  more  common 
than  a  single  attack.  I  found  among  104  cases  which  were 
analyzed  for  this  purpose  that  50  cases  had  one  attack;  17 
cases  had  three  attacks  ;  26  cases  had  two  attacks ;  7  cases 
had  four  attacks  ;  3  cases  had  five  attacks,  and  1  case  had 
eight  attacks.  Notwithstanding  this  tendency  to  relapses 
the  disease  is  an  eminently  curable  one.  It  is  only  in  a  few 
cases  that  the  disease  becomes  chronic,  as  in  a  patient  of 
Meldner,  who  developed  chorea  in  early  life  and  remained 
choreic  until  his  death,  at  the  age  of  sixty-six  years. 

The  interval  between  the  relapses  is  also  subject  to  great 
variation.  In  a  few  cases  the  relapse  may  set  in  after  sev-. 
eral  weeks  ;  in  others  after  several  years ;  and  in  the  case 
of  chorea  of  pregnancy  we  often  find  that  ten  years  or  more 
have  elapsed  since  the  preceding  attack.  The  female  sexr 
for  reasons  too  evident  to  mention,  is  more  prone  to  re- 
lapses than  the  male  sex.  The  second  and  third  attack  is 
generally  supposed  to  be  milder  than  the  first,  but  there 
are  exceptions  to  this  rule,  for  the  very  worst  cases  of 
chorea  that  I  have  ever  seen  have  been  in  patients  who 
were  passing  through  second  and  third  attacks.  Later 
attacks,  as  a  rule,  simulate  the  earlier  ones.  If  the  first 
attack   has   been   a  hemichorea   it   is   very   probable   that 


Il8  THE   NERVOUS  DISEASES    OF   CHILDREN. 

later  attacks  will  be  of  the  same  character.  The  severity 
of  development,  with  the  exceptions  just  mentioned,  is  very- 
much  as  in  the  first  or  in  the  earlier  attacks. 

Diagnosis. — The  diagnosis  of  chorea  rests  entirely  upon 
the  character  of  the  movements.  These  are,  as  a  rule,  un- 
mistakable, and  are  so  typical  that  when  the)7  occur  in  con- 
nection with  other  diseases  we  speak  of  such  movements  as 
"  choreic  "  or  "  choreiform."  In  practice  the  question  is 
most  frequently  raised  whether  a  child  is  suffering  merely 
from  general  nervousness  or  from  typical  chorea.  It  has 
been  my  habit  to  decide  this  point  not  merely  upon  the 
character  of  the  movements,  though  it  would  be  safe  enough 
to  do  this,  but  chiefly  upon  the  presence  of  other  symptoms, 
which  I  consider  of  still  greater  diagnostic  importance.  I 
refer  particularly  to  the  characteristic  movements  of  the 
tongue,  and  to  what  I  have  previously  alluded  to  as  the 
"  facies  "  of  chorea.  In  rare  instances  a  child  may  be  able 
to  imitate  the  choreic  movements  of  others  and  thus  simu- 
late true  chorea,  but  if  it  be  mere  simulation  the  attempt 
will  not  be  a  prolonged  one  nor  will  it  be  successful.  Hys- 
terical chorea  can  be  distinguished  very  readily  from  the 
chorea  of  Sydenham  by  the  more  rhythmical  character  of 
the  movements,  by  the  peculiarity  of  the  onset,  by  the 
longer  free  intervals  between  the  attacks  of  twitching,  by 
the  longer  duration  of  the  disease,  and  by  the  presence  of 
other  stigmata  of  hysteria. 

It  is  not  generally  appreciated  that  the  choreiform 
movements  associated  with  infantile  cerebral  palsies  are 
apt  to  be  mistaken  for  true  chorea.  This  post-hemiplegic 
chorea  is  very  similar  to  the  ordinary  form,  but  it  is  more 
strictly  unilateral ;  it  is  more  persistent,  and  it  is  invariably 
associated  .with  other  symptoms  which  prove  the  previous 
existence  of  paralysis.  The  difficulties  of  diagnosis  are  in- 
creased by  the  fact  that  in  every  case  of  severe  chorea 
there  is  more  or  less  weakness  of  the  affected  members,  but 
in  such  cases  I  would  advise  examination  for  the  existence 
of  contractures  and  for  increase  of  the  reflexes,  symp- 
toms which  are  characteristic  of  preceding  paralysis,  even 
though  there  be  little  actual  weakness  at  the  time.  Rigid- 
ity and  increased  reflexes,  moreover,  are  never  present  in 


CHOREA.  Wj 

cases  of  uncomplicated  functional  chorea.  As  this  post- 
hemiplegic chorea  is  as  much  the  expression  of  organic 
lesion  of  the  brain  as  is  post-hemiplegic  epilepsy,  it  is  nat- 
ural that  the  choreic  symptoms  should  continue  as  long  as 
the  cerebral  lesion  which  has  given  rise  to  them  continues 
in  force.  In  a  collection  of  cases  of  infantile  cerebral  pal- 
sies I  have  found  this  post-hemiplegic  chorea  present  in 
about  six  per  cent,  from  which  it  is  evident  that  choreic 
movements  are  not  nearly  so  frequently  found  in  conjunc- 
tion with  these  cerebral  diseases  as  athetoid  movements 
are.  I  have  been  consulted  a  number  of  times  for  persistent 
chorea,  and  in  several  such  cases  it  has  been  my  experience 
that  the  family  physician  has  overlooked  a  preceding  hemi- 
plegic  attack  which  could  have  been  determined  readily 
enough  if  attention  had  been  paid  to  the  existing  contract- 
ures and  to  the  increase  of  the  reflexes.  I  would  urge  that 
in  every  case  of  chorea  a  careful  examination  be  made  for 
other  evidences  of  organic  brain  trouble. 

Confounding  chorea  and  epileptiform  convulsions  is 
scarcely  conceivable,  for  the  convulsive  movements  of  epi- 
lepsy come  on  at  rarer  intervals,  there  is  generally  some 
momentary  loss  of  consciousness,  and  there  are  other  symp- 
toms pointing  to  epilepsy.  In  a  previous  publication  I  re- 
ferred to  the  child  of  a  colleague  who  would  make  sudden 
and  very  quick  twitchings  of  an  arm  and  of  a  leg.  If  these 
twitchings  occurred  while  the  child  was  walking  or  running 
across  the  room  it  would  stand  still,  evidently  surprised  by 
these  movements.  It  was  natural  to  think  of  petit  mal, 
but  the  frequency  of  the  movements,  the  character  of  the 
twitchings,  and  the  general  choreic  behavior  of  the  child 
helped  me  to  exclude  petit  mal  and  to  recognize  the  case 
as  one  of  true  chorea.  The  diagnosis  was  corroborated  by 
the  very  prompt  result  of  antichoreic  treatment. 

Morbid  Anatomy  and  Pathology.— In  considering 
this  part  of  the  subject  we  meet  with  very  much  the  same 
difficulties  which  we  encountered  with  regard  to  epilepsy, 
and  the  resemblance  between  the  two  is  also  a  close  one  in 
this  respect,  that  we  not  only  have  a  general  functional  dis- 
ease, which  in  the  one  case  we  call  epilepsy  and  in  the  other 
chorea  ;  but,  like  epilepsy,  chorea  is  also  frequently  enough 


120 


THE  NERVOUS  DISEASES    OF   CHILDREN. 


the  expression  of  actual  cerebral  disease.  It  is  natural 
therefore  to  infer  that  ordinary  chorea  must  be  due  to  dis- 
turbances similar  to  those  which  we  find  in  cases  of  organic 
lesion.  Almost  every  conceivable  change  in  brain  structure 
has  been  at  one  time  or  another  held  responsible  for  the  de- 
velopment of  chorea.     See  collected  84  cases  of  chorea  on 


Fig.  47. — Dilatation  of  Blood-vessels  in  the  White  Matter  of  the  Convolutions  of  a  very 
Chronic  and  Severe  Case  of  Chorea.     (Dana.) 

which  a  post-mortem  examination  had  been  made.  In  16 
no  changes  were  found  in  the  central  nervous  system,  in  32 
there  were  lesions  in  the  brain  and  in  the  nerve-centres  ;  in 
the  remainder  there  was  congestion  of  the  serous  mem- 
branes. Ogle,  Pye-Smith,  and  others  refer  to  a  hyperaemia 
of  the  brain  and  cord.  As  long  ago  as  1868  Steiner  report- 
ed upon  a  careful  examination  of  three  cases  of  chorea.  He 
found  cerebro-spinal  anaemia  and  some  connective-tissue 
proliferation  in  the  upper  part  of  the  spinal  cord  ;  conse- 


CHOREA.  J2I 

quently  he  considered  chorea  to  be  the  result  of  spinal  irri- 
tation. Meynert  and  Elischer  found  hyaline  degeneration 
in  the  nerve-cells  of  the  central  ganglia.  The  latter  author 
also  found  changes  in  the  vessels  of  the  central  ganglia  as 
well  as  extravasation  of  blood  into  the  connective  tissue  of 
the  brain,  and  also  numerous  emboli  in  the  smallest  vessels 
of  the  cortex.  He  described  peculiar  corpuscles — highly 
refractile  bodies — but  Wollenberg  has  found  them  in  the 
brains  of  non-choreic  patients.  Flechsig  has  found  hyaline 
changes  in  the  anterior  divisions  of  the  lenticular  nucleus. 
More  recently  Dana  has  observed  not  only  a  general  hyper- 
emia of  the  brain,  but  a  degeneration  in  the  walls  of  the 
blood-vessels,  in  the  white  substance  of  the  brain,  and  con- 
siderable perivascular  exudation  with  an  accumulation  of 
leucocytes.  Many  others,  chief  among  them  Hughlings 
Jackson,  have  insisted  upon  the  embolic  origin  of  chorea,  a 
theory  that  would  be  plausible  enough  since  Dickenson 
has  found  that  in  17  of  22  fatal  cases  endocarditis  was 
associated  with  the  chorea  ;  and  yet  this  theory  will  not 
explain  that  large  number  of  cases  in  which  there  is  no 
involvement  of  the  heart.  Furthermore,  an  examination  of 
the  brain  in  fatal  cases  of  chorea  by  competent  observers 
has  failed  to  reveal  the  presence  of  emboli.  This  view  of 
the  relation  between  capillary  embolism  of  the  brain  and 
chorea  was  suggested  by  Angel  Money,  who  noticed  after 
injections  of  a  fluid  into  the  carotids  of  animals  movements 
closely  resembling  those  of  chorea,  and  this  condition  was 
found  after  death  to  be  associated  with  capillary  embol- 
ism of  the  brain  and  cord. 

Lockhart-Clarke  found  changes  in  the  nerve-elements 
and  connective  tissue  in  the  spinal  cord ;  Garrod  speaks  of 
an  overgrowth  of  connective  tissue  in  the  nerve  centres, 
and  thus  we  might  go  on  quoting  as  many  different  find- 
ings as  there  are  authors  who  have  written  upon  this  sub- 
ject. One  of  the  later  contributions  to  this  subject  is  by 
Anton,  who  found  a  lesion  or  old  scar  in  the  outermost 
division  of  the  lenticular  nucleus.  As  the  same  lesion  was 
present  in  both  halves  of  the  brain,  and  the  chorea  was 
also  a  symmetrical  one,  the  author  is  inclined  to  attribute 
the  choreic  disease  to  these  lesions.     By  way  of  contrast 


122 


THE   NERVOUS  DISEASES   OE   CHILDREN. 


this  same  author  reports  the  case  of  a  man,  sixty-five  years 
of  age,  in  whom  spontaneous  and  associated  movements 
were  entirely  wanting  in  the  left  half  of  the  body.  In  the 
brain  of  this  man  the  thalamus  was  very  considerably  dis- 
eased, and  was  supposed  to  be  the  cause  of  the  defective 
movements.  I  need  not  discuss  the  author's  theory  at- 
tributing the  excessive  movements  to  the  disease  of  the 
lenticular  nucleus,  and  the  defective  movements  to  the 
thalamus.  Other  authors,  basing  their  conclusions  upon  a 
number  of  autopsies,  have  attributed  choreic  movements 
to  disease  of  the  thalamus. 


In  an  able  article  on  rabies  Golgi  refers  incidentally  to  his  studies  on 
cortical  changes  in  chorea,  which  were  published  in  1874,  and  have  been 
quoted  since  by  v.  Ziemssen 
and  others.  As  a  matter  of 
historic  interest  the  annexed 
figure  is  reproduced,  showing 
the  changes  in  the  nerve- 
cells  of  the  cerebellum  ;  Gol- 
gi also  reports  that  the  gan- 
glion cells  of  the  cortex,  and 
the  cells  of  Purkinje,  in  the 
cerebellum,  were  calcified.  It 
is  doubtful,  surely,  whether 
there  is  any  causal  relation 
between  these  changes  and 
others  pointing  to  a  chronic 
interstitial  encephalitis,  and 
the  disease  proper.  More- 
over, Golgi's  patient  died  at 
the  age  of  thirty-two  years,  and 
in  him  chorea  was  associated 
with  chronic  mental  disease. 

Up  to  the  present  time  the  results  of  bacteriological  research  are  not  very 
promising.  Berkeley  found  the  staphylococcus  pyogenes  aureus  in  cultures 
from  the  blood  of  a  fatal  case  of  chorea. 

Dana  has  published  the  history  and  autopsy  of  a  case  of  chronic  chorea. 
The  patient  was  thirty-four  years  of  age  at  time  of  death  ;  he  had  his  first 
attack  of  chorea  at  fourteen,  and  repeated  attacks  after  that.  The  post- 
mortem findings  included  a  chronic  lepto-meningitis  of  the  convexity  of  the 
brain,  hyaline  bodies  in  the  brain  cortex,  slight  meningitis  of  the  upper  part 
of  spinal  cord,  and  slight  meningo-encephalitis.  Diplococci  were  found  in 
the  proliferating  tissue  between  the  meninges  and  the  brain. 

The  case  is  of  unusual  interest,  showing  that  choreic  symptoms  may  be 


Fig.  48.— Changes  in  Purkinje's  Cells  in  Chorea ; 
Varicose  Swelling  of  the  Nerve-processes.  (Golgi. ) 


CHOREA.  123 

associated  with  a  wide-spread  affection ;  but  the  true  pathology  of  chorea  can- 
not be  made  out  in  any  case  of  fourteen  years'  standing,  nor  can  such  a  case 
be  relied  upon  to  prove  the  "  germ-theory  of  chorea."* 

The  only  just  inference  from  the  preceding  account  is 
that  the  accurate  pathology  and  morbid  anatomy  of  chorea 
are  still  unknown.     Of  the  changes  that  have  been  reported 
by  various  writers,  many,  if  not  most  of  them,  are  secondary 
and  not  primary.    All  that  we  can  claim   at  present  is  that 
there  is   considerable    change    in  the   gray  matter  of   the 
central  nervous  system  ;  that  the  entire  motor  tract  may  be 
involved,  but  that  the  changes  occur  more  frequently  in  the 
cortex  than  in  other  parts  of  the  brain.     These  choreiform 
movements  are  often  associated  with  gross  lesions  in  the 
cortex  ;  they  are  for  this  reason  more  common  in  the  child 
than  in  the  adult,  and  a  lesion  anywhere  in  the  brain  so 
situated  that  it  cuts  off  cortical  impulses  may  give  rise  to 
chorea.     The  occasional  development  of  mental  symptoms, 
the  association  of  chorea  and  epilepsy,  the  one  following 
upon  the  cessation  of  the  other,  are  the  symptoms  which 
not  only    indicate  a    cerebral  but    also  a   cortical   origin. 
With  more  recent  methods  of  examination,  such  as  those 
described  by  Golgi  and  Cajal,  we  may  be  able  to  make  out 
the  permanent  changes  in  the  nerve-elements  of  the  brains 
of  choreic  persons,  but  even  such  changes  may  be  second- 
ary  to  alteration  of  the  blood-supply  ;  in  fact  the  tendency 
of  the  present  day  appears  to  be  to  regard  chorea  as  due 
primarily  to  vascular  changes,  and  such  vascular  changes 
may  be  the  result  of  infection. 

Prognosis.— Complete  recovery  is  the  rule  in  the  ma- 
jority of  cases  of  chorea.  The  prognosis  may  be  a  little 
doubtful  in  regard  to  the  recurrence  of  the  disease  and  the 
duration  of  an  attack.  A  child  that  has  once  had  chorea 
has  acquired  a  distinct  predisposition  to  the  disease,  and 
often  an  occurrence  which  would  leave  no  impression  upon 
a  healthy  child's  nervous  system  is  sufficient  to  bring  about 
a  relapse  of  the  disease.     Later  attacks  are    generally  as 

*  Osier  refers  to  the  bacteriological  researches  of  Pianese  (Naples,  1893).  Animals 
inoculated  with  a  culture  of  bacillus  taken  from  a  choreic  patient  died  "  with  muscular 
twitchings  and  convulsions,"  and  the  same  bacillus  could  be  obtained  in  pure  cultures 
from  the  central  nervous  system.    But  it  is  doubtful  whether  these  animals  had  chorea. 


124  THE   NERVOUS  DISEASES    OF   CHIIDREN. 

mild  as  the  first,  and  there  is  no  special  reason  to  fear  an 
unfavorable  issue  in  later  attacks  unless  severe  complica- 
tions set  in.  Under  such  circumstances  not  the  chorea,  but 
the  complicating  rheumatism,  or  endocarditis,  is  the  actual 
source  of  danger.  Death  occurred  in  only  two  per  cent,  of 
the  cases  collected  by  the  British  Medical  Investigation 
Committee,  and  Sinkler  states  that  in  Philadelphia,  in 
seventy-four  years,  there  have  been  but  sixty-four  deaths 
from  chorea.  This  latter  statement  does  not  mean  very 
much,  as  the  majority  of  the  cases  of  chorea  ending  fatally 
would  be  reported  as  cases  of  one  of  the  complicating  con- 
ditions. 

There  is  no  way  of  predicting  positively  the  duration  of 
a  choreic  attack.  The  milder  an  attack  at  the  beginning 
the  more  likely  it  is  to  run  a  short  course,  whereas  the 
severer  forms  are  apt  to  be  much  more  chronic,  but  severe 
cases  under  proper  treatment  will  yield  much  more  quickly 
than  mild  cases  that  are  handled  improperly.  Under  com- 
petent medical  care  the  first  attack  may  be  recovered  from 
in  a  period  varying  from  four  to  ten  weeks  unless  serious 
complications  arise.  A  few  get  well  more  quickly,  but  they 
are  the  exceptions  rather  than  the  rule. 

Treatment. — A  severe  case  of  chorea  puts  the  skill  of 
the  attending  physician  to  a  severe  test,  while  in  a  mild 
case  the  less  medical  interference  the  better  for  the  child. 
Increased  experience,  both  in  private  and  in  dispensary 
practice,  prompts  me  to  urge  the  simple  plan  of  treatment 
which  I  outlined  a  few  years  ago. 

The  most  important  factor  in  the  treatment  of  chorea  is 
rest,  absolute  rest,  often  to  the  exclusion  of  all  other  thera- 
peutic measures.  Take  a  choreic  child  that  has  been  ac- 
customed to  roam  about  at  will  and  put  it  to  bed ;  it  will  be 
a  little  restless  for  the  first  few  days,  but  it  will  soon  quiet 
down  and  show  the  great  ad  vantage  of  a  thorough  rest  in  bed. 
There  is  difficulty  occasionally  in  carrying  out  this  plan,  for 
mothers  and  nurses  are  only  too  likely  to  be  disheartened 
by  the  first  show  of  resistance  on  the  part  of  the  child  and 
its  unwillingness  to  remain  quiet ;  but  with  a  few  exceptions 
this  morbid  restlessness  on  the  part  of  the  child  disappears 
within  a  few  days,  and  the  little  patient  feels  very  much 


CHOREA.  12$ 


happier  in  bed  than  out.  After  a  few  days  of  enforced  rest 
a  decided  improvement  is  noticeable.  In  the  milder  forms 
all  movements  cease,  and  in  the  severer  forms  the  child  is 
no  longer  troubled  by  the  annoying  jactations  of  the  limbs. 
According  to  the  severity  of  the  case  rest  should  mean  en- 
tire rest  in  bed  day  and  night.  If  the  disease  is  taking  a 
favorable  course,  after  a  week  or  two  the  patient  may  be 
taken  out  of  bed  for  half  an  hour,  an  hour,  or  two  hours, 
and  then  returned  again  for  the  remainder  of  the  day.  I 
have  met  only  very  few  cases  in  which  it  seemed  impossible 
to  carry  out  this  treatment ;  but  I  am  firmly  convinced  that 
it  was  never  the  fault  of  the  little  patient  but  always  the 
fault  of  incompetent  and  unintelligent  relatives.  If  the  dis- 
ease has  assumed  a  mild  form  we  can  endeavor  to  keep  the 
child  quiet  without  keeping  it  in  bed  the  entire  day  ;  a  few 
hours'  rest  will  be  better  than  none.  It  will  also  be  of  bene- 
fit to  the  child  to  forbid  its  taking  any  violent  exercise,  such 
as  running,  riding,  dancing,  or  bicycling. 

I  am  so  convinced  of  the  value  of  this  rest  treatment  in 
chorea  that  I  have  made  it  a  rule,  even  in  dispensary  prac- 
tice, to  insist  upon  this  point  of  rest ;  we  go  to  the  extent 
of  preferring  the  mothers  to  report  to  us  about  the  child 
rather  than  to  have  the  child  taken  out  of  bed  and  brought 
to  us  at  short  intervals. 

Next  in  importance  to  rest  is  a  nutritious  and  easily  di- 
gestible diet.  Milk  and  rest  will  do  more  for  most  cases  of 
chorea  than  any  other  two  measures.  The  nutritious  diet 
will  have  a  peculiarly  good  influence  upon  the  many  cases 
that  are  associated  with  profound  anaemia. 

The  monotony  of  this  special  form  of  rest-cure  can  be 
varied  with  advantage  by  the  use  of  lukewarm  baths.  Im- 
mersion into  a  cold  bath,  or  the  wet  pack,  with  subsequent 
friction,  cannot  be  recommended.  It  is  better  to  place  the 
child  in  lukewarm  water,  then  reduce  the  temperature  by 
adding  cold  water,  and  with  this  water,  that  is  growing 
colder  and  colder,  to  sponge  the  spine  thoroughly  in  order 
to  get  the  effect  of  the  dripping  water  upon  the  skin.  After 
the  bath  the  patient  should  be  kept  quiet  and  wrapped  up 
warmly.  In  every  case  proper  hygienic  and  dietary  meas- 
ures are  of  far  more  importance  than  medicinal  treatment, 


126  THE  NERVOUS  DISEASES    OF   CHILDREN. 

and  yet  we  are  bound  to  consider  the  various  drugs  that 
have  been  suggested  for  the  cure  of  this  disease. 

Among  these  arsenic  holds  the  first  place.  Its  praises 
have  been  warmly  sung  by  some,  while  others  have  decried 
it  as  but  little  better  than  any  other  drug  that  might  be 
substituted  for  it.  Only  a  few  years  ago  Dr.  Seguin,  in  his 
remarkably  lucid  lectures  on  the  various  forms  of  functional 
neuroses,  placed  arsenic  first  and  rest  second  in  the  treat- 
ment of  chorea.  This  order  I  think  should  be  reversed,  as 
I  have  yet  to  see  the  first  case  of  chorea  that  got  well  more 
quickly  with  arsenic  than  without,  as  long  as  it  was  getting 
the  benefit  of  rest.  Dr.  Seguin  insists  that  physicians, 
almost  without  exception,  give  nearly  useless  doses  of  ar- 
senic. He  regards  eighteen  to  twenty-seven  drops  of  Fow- 
ler's solution  after  each  meal  as  the  really  efficacious  dose. 
In  my  own  experience  very  few  children  will  tolerate  these 
large  doses,  which  should  at  all  times  be  given  in  some  alka- 
line water  within  an  hour  after  meals.  I  have  always  con- 
tented myself  with  smaller  doses,  varying  from  four  to 
twelve  drops  three  times  a  day,  and  if  the  cases  resisted 
treatment  I  prefer  abandoning  arsenic  rather  than  pushing 
it  to  the  extreme  which  Seguin  recommends.  In  cases  of 
excessive  restlessness  I  have  been  in  the  habit  of  prescrib- 
ing the  arsenic,  together  with  the  elixir  of  the  bromide  of 
sodium,  or  if  the  sleep  is  disturbed  I  give  the  evening  dose 
only  in  this  way  and  administer  the  arsenic  alone  during 
the  day  ;  the  free  use  of  chloral  and  bromides  is  to  be  con- 
demned. If  one  must  use  any  drug  in  the  cases  of  chorea, 
arsenic  is  to  be  preferred ;  but  it  cannot  be  expected  to  per- 
form miracles,  and  we  must  not  regard  it  in  any  sense  as  a 
specific  therapeutic  agent. 

Many  other  remedies  have  been  proposed,  all  have  been 
tried,  and  almost  all  have  been  abandoned.  For  some  time 
the  tincture  of  cimicifuga  was  in  great  favor.  From  fifteen  to 
thirty  drops,  three  times  daily,  may  be  administered  in  those 
cases  in  which  arsenic  is  not  well  borne  by  the  stomach.  I 
have  seen  no  good  reason  to  resort  to  the  use  of  hypodermic 
injections  of  arsenic,  as  recommended  by  Eulenburg  and 
Hammond ;  Weir  Mitchell  some  time  ago  proposed  the 
use  of  salicylates ;  Simon  and  Legroux  suggested  the  use 


CHOREA.  127 

of  antipyrin ;  and  the  oxides  and  sulphate  of  zinc  have  long 
been  in  popular  favor  in  the  treatment  of  this  disease  ;  but 
no  one,  I  think,  would  venture  conscientiously  to  recom- 
mend any  of  these  drugs  as  a  specific  against  chorea.  The 
preparations  of  iron  and  of  arsenic  fulfil  this  role  better  than 
any  other  drugs,  for  they  at  least  help  to  tone  up  the  gen- 
eral system.  Hirt  recommends  the  use  of  morphine.  Even 
if  this  drug  is  as  effective  as  it  is  claimed  to  be,  its  use  in 
young  children  is  not  to  be  encouraged.  In  the  earlier 
stages  of  chorea  it  is  essential  for  patients  to  obtain  sleep. 
According  to  the  age  of  the  child  chloral  in  five,  ten,  or 
fifteen-grain  doses  per  rectum  may  be  given.  If  it  is  neces- 
sary to  substitute  another  drug  I  would  suggest  the  use  of 
chloralamid,  of  trional,  of  veronal  (three  to  five  grains  at 
bedtime),  and  if  there  is  a  great  deal  of  mental  excitement 
I  should  favor  the  hypodermatic  use  of  the  hydrobromate 
of  hyoscine  (one  two-hundredths  to  one  one-hundredth 
grain). 

In  the  majority  of  cases  a  heart  tonic  will  be  necessary. 
Digitalis  in  drop  doses  of  the  fluid  extract  or  the  tincture 
of  strophanthus  should  be  given  in  cases  of  heart  weakness 
or  feeble  pulse.  Blaud's  pills,  the  sirup  of  the  iodide  of 
iron,  various  preparations  of  cod-liver  oil,  good  stimulating 
wines,  all  these  will  be  called  for  in  some  cases  of  chorea, 
but  whatever  else  one  may  be  induced  to  give  the  only 
matter  of  importance  is  that  absolute  rest  shall  be  enforced. 

Erb  has  advised  the  use  of  electricity.  A  weak  galvanic 
current  may  be  employed  safely  enough  and  may  be  applied 
to  the  nape  of  the  neck,  and  over  the  motor  areas ;  in  which 
case,  if  given  late  at  night,  the  current  will  help  to  bring  on 
normal  sleep.  A  moderate  stabile  current  of  from  fifteen  to 
twenty  cells  (about  ten  milliamperes)  applied  to  the  spine 
will  help  to  allay  restless  movements  of  the  body.  Massage 
may  be  given  in  some  cases  in  which  the  general  nutrition 
is  at  a  very  low  ebb  and  in  which  the  circulation  is  poor. 

A  special  caution  is  necessary  as  regards  the  question  of 
attendance  at  school.  Every  choreic  child,  however  mild 
its  attack  may  be,  should  be  kept  from  school  both  for  its 
own  sake  and  for  the  sake  of  the  other  pupils  who  might 
imitate  the  disease.     I  have  sometimes  allowed  myself  to 


128  THE  NERVOUS  DISEASES   OF  CHILDREN. 

be  persuaded  to  permit  a  child  with  a  mild  form  of  chorea 
to  continue  at  school ;  in  almost  every  instance  I  have  had 
reason  to  regret  it,  for  nothing  is  better  calculated  to  bring 
out  severe  chorea  than  the  competitive  spirit  that  obtains 
in  most  schools.  Periods  of  examination  are  fraught  with 
greatest  danger  to  those  children  who  have  had  attacks  in 
earlier  life.  The  atmosphere  of  the  school-room  seems  to 
have  a  depressing  influence  upon  such  children,  and  among 
the  wealthier  classes  far  better  progress  can  be  made  in  the 
ordinary  studies  if  the  child  is  instructed  at  home  than  if  it 
is  taught  at  school.  It  is  necessary  for  the  physician  to 
take  a  firm  stand  on  this  question  or  else  his  treatment  of 
the  case  will  be  thoroughly  unsatisfactory. 


CHAPTER  VI. 

CHOREIFORM   DISEASES. 

HEREDITARY   OR  HUNTINGTON'S  CHOREA. 

This  disease  is,  on  the  whole,  extremely  rare.  In  view 
of  its  hereditary  character  we  must  consider  it  in  connec- 
tion with  the  diseases  of  children,  although  it  generally 
appears  about  the  age  of  thirty  or  forty.  The  disease  was 
first  observed  by  Huntington,  a  Long  Island  physician,  and 
it  has  since  been  designated  by  his  name.  It  appears,  how- 
ever, that  another  New  York  physician,  Waters,  described 
the  disease  in  a  letter  to  Dunglison.  For  a  long  time  the 
disease  was  little  known  beyond  America.  Of  later  years 
it  has  been  observed  and  discussed  by  a  number  of  English, 
German,  and  French  authors.  The  disease  runs  in  families 
and  is  spread  from  one  community  to  another  in  connection 
with  the  migration  of  the  afflicted  families,  so  that  now,  ac- 
cording to  Gray,  there  are  a  number  of  communities  in 
America  in  which  the  disease  is  prevalent ;  but,  as  he  further 
states,  there  is  great  secrecy  maintained  with  regard  to  it, 
as  the  affliction  is  looked  upon  as  a  distinct  stigma  resting 
upon  the  family. 

The  disease  affects  several  members  of  the  same  genera- 
tion, but  may  skip  as  many  as  it  selects.  The  descendants 
of  healthy  members  of  a  family  enjoy  immunity  from  the 
disease  as  a  rule.  In  King's  case,  which  I  quote  on  the  au- 
thority of  Gray,  the  great-grandfather  was  choreic.  He  had 
ten  children,  but  only  four  were  afflicted  with  this  disease; 
they  also  had  children  who  were  choreic.  One  of  these  four 
had  nine  children,  eight  of  whom  were  healthy,  but  the 
ninth  was  choreic.  The  ninth  had  five  children,  of  whom 
four  were  choreic.  Of  these  four,  three  had  no  children, 
but  the  fourth  had  a  chorea  whilst  he  was  still  young  and 


130  THE  NERVOUS  DISEASES    OF   CHILDREN. 

was  cured  of  it,  when  again  at  thirty-five  he  passed  gradu- 
ally into  the  typical  Huntington's  chorea. 

Symptoms. — The  disease  begins,  as  a  rule,  between  the 
ages  of  thirty  and  forty  years,  though  at  least  a  dozen  or 
more  cases  are  on  record  which  have  begun  before  the  age 
of  twenty.  Males  and  females  seem  to  be  afflicted  with 
the  disease  in  equal  numbers.  The  chief  symptom  is  a 
motor  disturbance,  which  for  lack  of  a  better  name  is  termed 
choreic,  yet  it  is  very  different  from  the  twitchings  of 
chorea  minor.  The  muscular  movements  of  Huntington's 
disease  are  coarse  and  grimacing,  and  may  be  distributed 
over  a  large  area  of  the  body.  In  Sydenham's  chorea  the 
affection  is  more  strictly  localized.  In  the  former  disease 
grimaces  and  all  sorts  of  extravagant  posturing  are  much 
more  pronounced  than  those  which  we  observe  in  the 
ordinary  chorea  of  children.  The  difference  in  degree  of 
muscular  activity  is  so  very  great  that  one  naturally  doubts 
the  connection  between  the  two  diseases.  As  a  rule,  the 
movements  are  slight  at  the  start,  affecting  the  face  and 
upper  extremities  only.  In  the  course  of  years  they  in- 
crease in  intensity,  and  become  widely  distributed  over  the 
entire  body,  until  in  the  end  every  single  muscle  or  group 
of  muscles  may  be  involved. 

The  muscular  disturbance  is  in  part  subject  to  the  will, 
and  patients  afflicted  with  this  disease  can,  if  they  make  a 
serious  effort,  inhibit  the  twitchings  very  much  better,  and 
for  a  much  longer  period,  that  those  suffering  from  ordinary 
chorea  can.  The  motor  power  is,  as  a  rule,  not  diminished, 
but  on  account  of  the  irregularity  of  muscular  actions  it  is 
extremely  uncomfortable  for  the  patient  to  walk  about,  and 
he  easily  becomes  an  object  of  pity  or  of  ridicule. 

There  are  no  sensory  disturbances,  and  the  reflexes, 
while  they  may  vary  much,  are  not  morbidly  exaggerated 
or  diminished.  By  far  the  most  characteristic  symptom  of 
Huntington's  chorea  is  the  association  with  it  of  a  progres- 
sive dementia.  The  first  sign  of  a  mental  change  may  be 
a  simple  depression  which  is  deepened  by  a  knowledge 
of  the  hereditary  and  incurable  character  of  the  disease. 
Under  the  circumstances  the  occurrence  of  suicide  is  not 
unnatural.    The  mental  condition  at  the  start  varies  a  little  ; 


HEREDITARY   OR   HUNTINGTON'S   CHOREA.  J  3  I 

the  patient  may  be  either  irritable  or  apathetic.  In  the 
course  of  time  the  deterioration  of  all  the  mental  faculties 
is  very  marked  and  a  typical  dementia  is  developed.  Speech 
is,  as  a  rule,  thick  and  indistinct,  sometimes  nasal  and  con- 
fused. Unless  some  intercurrent  disease  puts  an  end  to 
life  these  patients  may  linger  on  for  many  years,  may  be- 
come absolutely  bedridden,  a  burden  to  themselves  and  a 
torment  to  their  families. 

The  course  of  the  disease  is  a  very  chronic  one,  and 
unfortunately  does  not  tend  to  shorten  human  life.  Sev- 
eral of  those  who  have  exhibited  the  first  symptoms  of  dis- 
ease at  the  age  of  thirty  have  lived  to  sixty  and  seventy 
years.  Whether  cases  beginning  very  early  in  life  live 
as  long,  cannot  be  distinctly  stated,  but  there  is  nothing 
improbable  in  supposing  that  they  do. 

Etiology. — Among  the  causes  of  this  disease  none  is 
more  potent  than  heredity.  It  is  distinctly  a  family  dis- 
ease, and  unlike  other  such  diseases  it  does  not  seem  to 
skip  a  generation.  Persons  with  this  disease  can  generate 
healthy  children  whose  descendants  may  not  be  afflicted 
with  this  trouble.  To  all  appearances  an  exciting  cause  is 
needed  to  develop  the  disease.  In  many  of  the  cases  the 
first  symptoms  appear  upon  severe  emotional  excitement 
or  after  some  acute  disease.  In  this  respect  the  resem- 
blance between  the  hereditary  chorea  and  chorea  minor 
is  very  striking.  Rheumatism  does  not,  however,  play  the 
part  in  the  hereditary  form  that  it  does  in  ordinary  chorea. 

Differential  Diagnosis. — The  strong  factor  of  hered- 
ity and  the  appearance  at  a  relatively  advanced  age  are 
sufficient  to  distinguish  these  cases  from  ordinary  chorea. 
There  is  some  danger,  however,  of  confusing  these  cases 
with  the  post-hemiplegic  chorea  that  is  developed  early  in 
life,  and  this  danger  is  all  the  greater  if  the  onset  of  the 
paralytic  symptoms  is  uncertain,  or  if  the  paralysis  has 
disappeared  and  the  chorea  remains.  From  Friedreich's 
ataxia  the  disease  can  be  easily  differentiated  by  the  fact 
that  in  the  former  malady  the  disturbance  of  motion  is 
truly  ataxic  and  not  choreic,  and  the  reflexes  are  totally 
absent,  whereas  in  Huntington's  disease  the  reflexes  are 
not  markedly  altered.     In  Friedreich's  disease  there  is  also 


132  THE   NERVOUS  DISEASES    OF   CHILDREN. 

an  ataxic  gait,  made  worse  by  closing  of  the  eyes,  whereas 
in  Huntington's  disease  nothing  of  the  sort  is  observed. 
Some  forms  of  hysterical  chorea  might  be  confounded  with 
this  disease,  particularly  if  on  inquiry  the  statement  is  elic- 
ited that  a  similar  affliction  has  been  observed  in  other 
members  of  the  family,  and  it  should  not  be  forgotten  that 
the  hereditary  trouble  may  be  of  an  hysterical  order.  The 
examination  of  the  patient  and  the  general  hysterical  tem- 
perament, the  fact  that  the  choreic  movements  come  on  in 
the  nature  of  attacks,  and  the  determination  of  other  hys- 
terical symptoms  will  help  to  distinguish  one  disease  from 
the  other. 

Pathological  Anatomy. —  The  morbid  changes  in 
Huntington's  chorea  have  not  yet  been  definitely  made 
out.  Innumerable  changes  have  been  reported  by  various 
authors.  These  include  pachymeningitis,  hasmatoma,  tu- 
mors of  the  dura,  general  atheroma,  atrophy  of  the  motor 
convolutions,  increased  fluid  in  the  ventricles,  foci  of  soft- 
ening in  various  parts,  even  in  the  ganglia.  The  most 
frequent  states  are  pachymeningitis  and  changes  in  the 
cortical  tissue.  In  a  case  of  Charcot's  the  meninges  were 
adherent  in  certain  places,  and  the  cortical  substance  was 
evidently  sclerotic.  But  these  varying  conditions  are 
largely  secondary  processes  and  do  not  in  any  way  explain 
the  true  pathology  of  the  disease.  Dr.  Osier  summarizes 
the  changes  in  a  series  of  sections  from  the  brain  and  cord 
which  he  examined  as  follows :  "  The  arteries  were  thick- 
ened, and,  in  places,  show  hyaline  degeneration,  and  in  the 
smaller  arterioles  the  fatty  changes  were  very  marked  in 
the  fresh  specimens  from  the  cortex.  The  perivascular 
lymph-spaces  were  large,  and  contained  leucocytes.  The 
ganglion  cells,  in  many  sections,  showed  very  slight  changes, 
not  more  than  are  often  seen  in  chronic  cases  associated 
with  atrophy  of  the  convolutions.  There  was  the  common 
vacuolation,  and  many  cells  seemed  laden  with  pigment. 
The  increase  in  the  connective-tissue  elements  was  more 
evident  to  the  touch  and  upon  section  than  microscopically. 
Sections  of  the  pons  and  medulla  showed  no  foci  of  disease. 
Beyond  the  thickening  of  the  arteries  and  the  shrinkage 
of  the  cells  of  the  anterior  cornua — probably  an  .artificial 


HEREDITARY  CHOREA    WITHOUT  DEMENTIA.  I  33 

change — the  sections  of  the  cord  showed  no  important  le- 
sions."" Oppenheim,  Hoppe  and  others  found  dissemi- 
nated, miliary  encephalitis  of  the  cortex.  Binswanger 
thinks  the  morbid  changes  not  unlike  those  of  general  pare- 
sis. French  authors  attach  great  importance  to  the  pro- 
liferation of  the  neuroglia  of  the  cortex. 

Prognosis. — The  prognosis  of  Huntington's  chorea  is 
grave  as  regards  the  cure  of  the  trouble,  and  in  view  of  the 
marked  dementia  which  is  associated  with  almost  every  case. 
Unfortunately,  however,  it  is  not  a  fatal  disease,  and  patients 
so  afflicted  may  live  on  to  a  very  old  age. 

Treatment. — As  for  the  treatment  of  these  cases  the 
same  principle  should  be  observed  as  in  ordinary  chorea — 
rest,  freedom  from  care,  and  excitement,  separation  from 
family,  and  change  of  climate,  and  possibly  a  course  of 
arsenic  treatment  may  be  of  some  benefit.  If  movements 
are  excessive  and  the  sleep  of  the  patient  is  unsatisfactory, 
the  exhibition  of  hyoscine,  in  doses  of  one  one-hundredth  of 
a  grain,  or  of  trional  or  chloralamid,  in  ten-  to  twenty-grain 
doses,  once  or  twice  a  day,  is  worthy  of  a  trial.  But,  in  the 
nature  of  things,  every  form  of  treatment  will  be  simply 
palliative. 

HEREDITARY   CHOREA   WITHOUT   DEMENTIA. 

There  is  another  form  of  chorea  of  which  a  slight  mention  should  be  made 
in  this  connection.  The  disease  has  a  distinct  hereditary  tendency,  and  is 
either  transmitted  direct  from  parent  to  child  or  from  a  more  remote  ances- 
tor. The  disease  appears  early,  beginning  as  a  rule  at  about  the  age  of 
puberty  and  continuing  during  life.  It  is  characterized  by  distinct  choreic 
movements  of  the  hands  and  tongue  and  the  facial  muscles.  The  move- 
ments may  become  so  marked  that  they  interfere  with  every  voluntary  effort, 
such  as  riding,  sewing,  buttoning  of  clothes,  and  the  like.  The  lower  ex- 
tremities do  not  become  affected,  and  the  general  health  of  the  patient  is  not 
much  impaired,  except  that  the  annoyance  of  the  disease  may  bring  on  a 
despondent  feeling  ;  and  in  one  case  that  has  come  under  my  notice  distinct 
melancholy  has  occurred.  It  is  distinguished  from  Huntington's  chorea 
above  all  by  the  entire  absence  of  symptoms  pointing  to  dementia.  The 
persons  afflicted  with  this  form  remain  bright  throughout  life  and  are  able  to 
attend  to  their  ordinary  affairs.  It  is  furthermore  to  be  distinguished  from 
chronic  chorea  from  the  fact  that  there  is  no  distinct  history  of  an  acute 
attack  after  which  the  chorea  has  been  developed ;  that  the  choreic  move- 

*  Greppin  claims  that  the  disease  is  due  to  a  morbid  process  (not  unlike  an  encepha- 
litis), which  is  developed  gradually  and  in  various  parts  of  the  brain. 


134  THE   NERVOUS  DISEASES   OF   CHILDREN. 

merits  have  never  been  as  wide-spread  as  they  are  in  such  cases  of  chorea 
minor  as  develop  into  chronic  chorea.  They  generally  begin  a  little  later  in 
life,  too,  than  the  acute  chorea  minor  does,  and  there  is  no  distinct  history  of 
individual  attacks.  If  the  disease  has  once  been  established  it  continues 
without  any  marked  remission,  but  also  without  any  rapidly  progressive 
changes.  Some  confusion  might  also  arise  with  the  post-paralytic  chorea, 
but  under  such  circumstances  an  examination  into  the  past  history  of  the 
patient,  and  for  the  evidences  of  persistent  paralysis,  will  help  to  clear  up  the 
diagnosis. 

The  prognosis  is  entirely  favorable  as  regards  life,  but  less  can  be  said  as 
regards  the  cure  of  the  disease.  I  have  placed  such  patients  under  the  com- 
plete rest-cure,  and  have  for  a  time  obtained  distinct  improvement  in  the 
choreic  movements.  General  tonic  measures  should  be  employed,  and  the 
proper  feeding  of  the  patients  carefully  looked  after. 

HABIT   CHOREA. 

It  is  well  known  that  children  are  subject  to  "tricks"  of  movements. 
These  include  simple  or  co-ordinated  movements  of  various  muscles,  gen- 
erally of  the  muscles  of  the  face,  of  the  eyes,  of  the  shoulder,  and  even  of  the 
thighs.  These  "  habit  spasms  "  or  "  tics  "  may  resemble  chorea ;  often  the 
jerkings  are  so  violent  in  character  as  to  suggest  "  epileptoid  "  rather  than 
"  choreic  "  disease. 

It  is  not  always  an  easy  matter  to  determine  how  the  "  trick  "  was  en- 
gendered ;  in  some  instances  a  true  chorea  has  been  the  starting-point,  the 
children  keeping  up  some  form  of  twitching  movement  after  the  St.  Vitus's 
dance  has  disappeared.  Through  imitation,  habit  chorea  may  be  developed 
in  children  who  have  watched  others  with  chorea  minor. 

A  habit  chorea  developed  in  early  years  is  often  continued  throughout 
life.  Among  professional  men  (artists,  literary  men,  and  even  physicians) 
such  "  tricks  "  are  not  uncommon,  and  the  doubt  arises  whether  serious  ef- 
fort has  been  made  to  dispel  the  habit.  Among  the  commoner  forms  of 
"  habit  chorea  "  are  blinking,  facial  contortions,  sniffing,  shrugging  of  the 
shoulders,  or  some  trick  in  speech  or  gesture.  A  friend  of  mine,  now  a  well- 
known  astronomer,  has  since  boyhood  never  answered  a  question  without 
first  saying,  Hm  ?  ah  ?  eh  ?  In  former  years  this  utterance  was  accompanied 
by  an  exaggerated  raising  of  the  eyebrows,  as  if  to  intensify  the  interrogation. 

Such  habits  cannot  be  condemned  sufficiently  in  children.  Parents 
should  discipline  children  severely  in  order  to  rid  them  of  the  habit. 

If  there  is  any  peripheral  condition  (such  as  nasal  obstruction  or  eye- 
strain) which  helps  to  keep  up  the  habit,  the  condition  should  be  treated 
carefully.  It  is  important  also  to  make  certain  that  the  "  habit  spasm  "  is 
not  true  chorea.  This  can  be  done  by  examining  for  other  symptoms  of  St. 
Vitus's  dance.  The  general  condition  of  children  with  some  form  of  habit 
spasm  may  require  treatment ;  many  of  them  are  anasmic  or  scrofulous. 

Complex  Co-ordinated  Movements  (Complex  Tics). — In  children 
as  well  as  in  adults,  complex  movements  of  a  definite  character  are  repeated 


COMPLEX   CO-ORDINATED   MOVEMENTS.  135 

at  intervals,  or  may  be  continuous.  They  bear  only  a  very  slight  resemblance 
to  chorea;  but,  as  a  matter  of  convenience,  they  may  be  discussed  in  connec- 
tion with  "habit  chorea."  Some  of  them,  the  gyrospasms,  for  instance, 
occur  in  very  young  infants  ;  others  occur  in  older  children  and  are  frequently 
associated  with  slight  mental  derangement.  Some  authors  have  included 
them  under  the  heading  of  Imperative  Movements,  and  have  described  them 
as  head-shaking,  head-rotating,  head-banging,  head-nodding,  etc.  These 
movements  are  often  associated  with  nystagmus  and  with  defective  mental  de- 
velopment, thus  indicating  that  in  some  instances  they  may  be  due  to  organic 
changes  in  the  brain.  These  peculiar  disorders  have  been  studied  carefully 
by  Henoch,  Hadden,  Gee,  Peterson,  and  Mills,  and  have  been  referred  to  by 
Osier  and  others. 

Gyrospasms  of  the  Head  is  a  term  applied  by  Peterson  to  peculiar  ro- 
tary movements  of  the  head  in  children,  associated  at  times  with  strabismus 
or  nystagmus.  The  movements  of  the  eye  are  very  rapid  (Risien  Russell) 
and  may  affect  one  eye  only ;  the  nystagmus  may  be  of  the  convergent  order 
(J.  Thomson).  Two  of  Peterson's  five  cases  were  observed  by  him  in  my 
clinic.  Both  these  cases  were  in  young  infants,  eight  and  nine  months  of  age 
respectively  ;  in  one  the  movements  came  on  after  a  fall ;  in  the  other  there 
was  no  history  of  traumatism.  The  chief  points  of  Case  II.,  as  described  by 
Peterson,  are  as  follows:  The  child  was  nine  months  old  when  examined. 
Since  the  age  of  four  months  he  had  a  rotary  movement  of  the  head.  When 
the  child  was  quiet,  the  head  kept  oscillating  from  right  to  left  and  from  left 
to  right  at  the  rate  of  eighty  to  one  hundred  oscillations  per  minute.  The 
movement  ceased  at  times,  particularly  if  the  child's  gaze  was  riveted  upon 
some  object.  There  was  lateral  nystagmus  of  the  right  eye,  also  ceasing 
for  minutes  at  a  time. 

These  gyrospasms,  which  are  evidently  of  a  piece  with  head-nodding, 
head-jerking,  etc.,  are  scarcely  to  be  confounded  with  epilepsia  nutans.  If 
developed  in  later  years,  they  may  constitute  a  habit,  but  if  the  movements 
begin  in  early  infancy  and  are  associated  with  nystagmus,  strabismus,  or 
idiocy,  one  need  not  hesitate  to  ascribe  them  to  cerebral  disease.  The  re- 
gion of  the  cranial-nerve  nuclei  would  be  the  most  probable  seat  of  the 
trouble  ;  and  the  lesion  must  be  supposed  to  be  irritating  in  character. 

The  chief  etiological  factors  appear  to  be  rickets,  intestinal  irritation,  and 
dentition.  The  prognosis  is  good  on  the  whole,  except  when  these  move- 
ments are  associated  with  idiocy.  A  mild  course  of  bromides  has  proved 
satisfactory  in  the  majority  of  cases.  Mills  recommends,  in  addition,  the  use 
of  two  or  three  minims  of  tincture  of  belladonna,  or  one  minim  of  the  fluid 
extract  of  conium. 

Imperative  movements  associated  with  arithmomania  (repeating  every- 
thing a  definite  number  of  times)  are  clearly  the  result  of  mental  derange- 
ment, and  do  not  seem  to  me  properly  to  belong  to  this  group  of  cases. 

Several  other  motor  neuroses  bearing    a  more  or  less 
close  resemblance  to  chorea  minor  have  been  described  in 


I36  THE  NERVOUS  DISEASES   OF   CHILDREN. 

connection  with  the  chief  disease.  While  the  cases  may 
have  a  superficial  resemblance  to  St.  Vitus's  dance  they 
are,  as  a  rule,  of  a  much  more  explosive  character.  They 
occur  in  families  with  a  neurotic  heredity,  and  represent, 
on  the  whole,  a  more  serious  disturbance  of  the  central 
nervous  system.  It  is  not  an  easy  task  properly  to  classify 
all  these  conditions.  There  has  been  much  confusion  in 
the  discussion  of  these  subjects,  and  no  two  authors  entirely 
agree  in  the  designation  of  these  disorders.  As  a  majority 
of  them  come  on  later  in  life,  or  at  least  are  fully  developed 
in  later  years,  we  need  not  in  this  book  treat  them  in  a  very 
exhaustive  manner. 

CHOREA   ELECTRICA. 

It  is  certain  that  two  distinct  forms  have  been  described  under  this  term. 
Henoch  includes  under  it  a  form  of  choreic  disturbance  which  resembles  in 
part  what  is  commonly  designated  as  habit  spasm,  but  some  of  his  cases  are 
more  distinctly  allied  to  myoclonia  or  paramyoclonia  multiplex.  Bergeron  al- 
ludes to  violent  choreic  movements  occurring  chiefly  in  children  between  the  ages 
of  seven  and  fourteen  years.  Bruns  considers  these  two  forms  to  be  identical. 
Oppenheim  is  inclined  to  label  Bergeron's  form  as  an  hysterical  chorea. 

The  term  electric  chorea  should,  possibly,  be  restricted  entirely  to  a  very 
rare  disease  that  occurs  chiefly  in  the  northern  part  of  Italy.  I  have  had 
opportunity  to  see  one  case  of  this  description  in  Italy,  and  one  in  an  Ital- 
ian woman  who  came  to  my  clinic.  The  condition  was  first  described  by 
Dubini,  in  1846,  and  to  avoid  confusion  it  would  be  as  well  to  speak  of  it  as 
Dubini's  disease.  The  disease  is  apt  to  occur  in  boys  and  girls  as  well  as  in 
men  and  women  of  advanced  years.  It  has  been  supposed  to  be  due  to  some 
infectious  agent,  but  the  rarity  of  the  disease,  even  in  those  districts  in  which 
it  does  occur,  would  seem  to  militate  against  this  view. 

The  chief  symptom  of  the  disease  is  a  series  of  violent  spasmodic  move- 
ments, affecting  particularly  the  muscles  of  the  neck  and  head,  as  well  as  of 
the  extremities.  Some  of  the  cases  begin  with  movements  in  an  arm,  which 
spread  to  the  leg  of  the  same  side,  and  finally  involve  the  opposite  half  of 
the  body,  and  also  the  trunk,  neck,  and  head.  After  some  months  the 
choreic  members  of  the  body  become  paralyzed ;  there  is  wasting  of  the  mus- 
cles and  a  loss  of  faradic  irritability.  Epileptiform  seizures  occur,  and  these 
may  either  be  partial  or  general.  In  the  course  of  a  year  or  more  the  pa- 
tient may  become  entirely  paralyzed,  and,  as  in  the  case  I  saw,  the  patient 
was  confined  to  his  bed,  unable  to  move  a  limb,  while  the  severest  choreic 
jactation  continued  in  the  muscles  of  the  neck.  The  majority  of  these  cases 
end  fatally  within  a  few  weeks  or  months.  During  the  course  of  the  disease 
the  patient  suffers  a  great  deal  from  pain,  has  slight  elevation  of  tempera- 
ture, but,  as  a  rule,  does  not  have  loss  of  consciousness. 


MA  LA  DIE  DBS  TICS   CONVULSIFS.  \$7 

The  disease  is  so  rare  that  sufficient  pathological  examinations  have  not 
been  made  to  warrant  definite  statements  with  regard  to  its  nature.  It  is 
probable  that  it  is  a  form  of  cerebro-spinal  disease,  as  is  indicated  by  the  con- 
vulsions on  the  one  hand  and  the  paralysis  with  atrophy  on  the  other. 

MALADIE   DES   TICS   CONVULSIFS. 

This  disease,  also  known  as  Gilles  de  la  Tourette's  disease,  has  been 
studied  most  carefully  by  Gilles  de  la  Tourette,  Guinon,  Brissaud,  Meige,  and 
Feindel  (who  have  written  a  monograph  on  the  subject),  Oppenheim  and 
others.  The  chief  symptoms  are  convulsive  twitchings  of  the  facial  muscles 
and  other  regular  systematic  movements,  explosive  conditions  of  speech 
known  as  echolalia  and  coprolalia,  and  lastly,  imperative  conceptions  and 
impulses.  The  disease,  as  a  rule,  appears  in  children  between  the  ages  of 
seven  and  fifteen  years.  It  is  more  apt  to  occur  in  those  predisposed  by 
inheritance  to  neurotic  troubles  than  in  others.  In  many  instances  there  is  a 
very  distinct  history  of  heredity,  the  same  disease  occurring  in  succeeding 
generations.     (Charcot,  Gintrac,  etc.) 

The  first  symptoms  that  appear  are  sudden  and  explosive  twitchings 
of  the  muscles  of  the  face  or  of  the  eyes.  The  mouth  may  be  twisted,  and 
all  sorts  of  grimaces  may  constitute  the  early  symptoms.  In  a  case  which 
I  have  had  under  observation,  the  boy  would  begin  by  making  grimaces 
and  then  turn  about  quickly  as  though  he  was  snapping  at  some  one. 
These  movements  were  performed  in  regular  succession.  Movements  of  the 
sterno-cleido  mastoid,  and  of  the  trapezius  are  often  repeated  so  systemat- 
ically that  they  seem  to  be  purposive.  The  child  may  at  the  same  time  begin 
to  spit  or  to  blubber,  and  the  entire  combination  of  symptoms  suggest  the 
possibility  of  simulation  or  intention,  particularly  if  jumping  or  leaping  move- 
ments are  associated  with  the  other  symptoms.  Whatever  the  symptoms 
may  be  that  have  been  developed  in  a  given  case  they  are  persisted  in  with 
remarkable  regularity,  and  can  be  distinguished  from  intentional  movements 
by  the  fact  that  the  patient  is  evidently  surprised  by  the  suddenness  and  vio- 
lence of  the  movements.  Smacking,  hissing  sounds  are  sometimes  heard, 
but  none  of  the  symptoms  is  more  characteristic  than  the  habit  of  repeating 
words  or  sounds  that  are  heard  (echolalia),  or  the  involuntary  sudden  explo- 
sive utterance  of  foul  language  (coprolalia).  A  little  patient  of  mine  uttered 
the  worst  curses  I  ever  heard,  which  she  had  evidently  picked  up  on  the 
street,  but  would  regret  the  utterance  the  very  next  moment  after  they  had 
passed  her  lips,  and  would  contritely  declare  that  she  was  entirely  irrespon- 
sible for  the  same. 

The  symptoms  frequently  continue  for  years,  and  while  the  intelligence  of 
the  person  so  afflicted  does  not  show  any  deficiency  only  a  few  are  able  to 
become  masters  over  these  explosive  seizures.  In  some  an  attempt  to  con- 
quer the  disease  produces  great  restlessness  and  general  excitement.  In 
others  the  symptoms  become  manifestly  worse  under  the  effort  to  control 
them,  and  it  is  advisable  under  such  conditions  to  divert  the  attention  of  the 
patient  from  his  symptoms  as  much  as  possible. 

Imperative  conceptions  are  frequently  associated  with  the  motor  symp- 


138  THE  NERVOUS  DISEASES    OF   CHILDREN. 

toms,  and  usually  seem  to  represent  a  psychic  explosion,  the  equivalent  of  the 
physical  symptoms.  Some  of  the  children  feel  compelled  to  utter  words  in  a 
definite  sequence,  to  pronounce  certain  letters  in  a  peculiar  way  ;  rolling  the 
"  r  "  and  making  a  hissing  "  s  "  are  particularly  frequent.  In  others  impera- 
tive actions,  such  as  occur  in  connection  with  neurasthenic  disorders,  are  quite 
common.  They  are  compelled  to  do  their  sums  over  and  over  again,  to  re- 
trace their  steps,  to  pick  up  everything  they  see  lying  on  the  street  or  in  the 
room,  and  are  compelled  to  be  in  a  state  of  continued  activity  in  consequence 
of  these  imperative  impulses. 

The  course  of  the  disease  is  extremely  chronic  ;  by  some  it  is  considered 
to  be  absolutely  incurable.  But  this  is  too  extreme  a  view,  since  some  of  the 
cases  get  well. 

The  prognosis  should,  however,  be  studied  carefully  in  each  case,  as  there 
is  no  telling  in  advance  whether  the  patient  will  respond  favorably  or  not  to 
the  treatment.  The  resemblance  between  this  disease  and  other  choreiform 
neuroses  is  at  times  so  close  a  one  that  a  differential  diagnosis  becomes  ex- 
tremely difficult.  It  can  hardly  be  confounded  with  ordinary  chorea  since 
the  movements  are  much  more  systematic  and  more  intermittent  than  they 
are  in  St.  Vitus 's  dance.  Moreover,  echolalia  and  coprolalia  never  occur  in 
ordinary  chorea.  The  difficulties  of  speech  in  chorea  are  the  very  reverse  of 
the  explosive  speech  in  this  maladie  des  tics.  Hysteria  and  hysterical  chorea 
may  bear  a  superficial  resemblance  to  this  disease  ;  but,  on  the  one  hand,  the 
stigmata  of  hysteria  are  generally  wanting,  and  in  hysteria  there  is  no  such 
constant  repetition  of  more  or  less  convulsive  movements  as  there  is  in  this 
disease.  There  is,  no  doubt,  a  close  resemblance  between  the  maladie  des 
tics  and  the  jumpers  and  many  other  forms  of  disease  which  are  differently 
designated  according  to  the  peculiar  variety  of  muscular  action.  The  one 
point,  however,  which  the  maladie  des  tics  has  in  common  with  other  similar 
conditions  is  its  occurrence  in  neurotic  families.  The  distinction  between 
maladie  des  tics  and  para-myoclonia  will  be  evident  after  a  discussion  of  the 
latter  trouble. 

Treatment. — The  most  important  factor  in  the  treatment  of  these  pa- 
tients is  their  complete  isolation  from  all  other  children  and  from  their  usual 
surroundings.  They  should  be  subjected  to  a  moderately  severe  discipline  by 
a  competent  nurse  or  parent,  and  should  be  taught  self-control  as  far  as  that 
may  be  possible.  The  mere  seclusion  from  other  persons  generally  serves  to 
lessen  the  excitement  and  the  number  of  explosions.  If  the  disease  has  led 
to  a  condition  of  exhaustion  from  loss  of  sleep  or  from  inanition  it  is  well  to 
secure  quiet  during  a  considerable  part  of  the  entire  day.  This  can  be  done 
best  by  the  administration  of  chloral  or  of  small  doses  of  morphine.  I  can- 
not see  the  wisdom  of  using  chloroform,  as  has  been  suggested  by  some.  If 
the  motor  restlessness  is  extreme,  hypodermic  injections  of  hydrobromate  of 
hyoscine,  in  doses  of  one  one-hundredth  grain,  carefully  administered,  may 
prove  of  some  benefit ;  the  ordinary  nerve-tonics,  such  as  arsenic  and  quinine, 
will  do  little  good,  and  it  is  best  not  to  waste  much  time  in  administering 
these  drugs.  I  am  confident  that  if  any  good  is  to  be  accomplished  we  must 
depend  entirely  upon  isolation,  proper  feeding,  and  the  use  of  tonic  hydro- 


THOMSEWS  DISEASE.  1 39 

therapeutic  procedures.     Regular  gymnastic  or  calisthenic  exercises  should 
be  tried  and  may  be  of  advantage. 

THOMSEN'S   DISEASE   (MYOTONIA  CONGENITA). 

In  connection  with  the  various  forms  of  disordered  movements  which 
have  been  considered  in  this  chapter,  we  may  discuss  in  a  very  brief  way  the 
disease  known  by  the  name  of  the  physician  who  gave  the  first  thorough 
description  of  it  as  it  occurred  in  his  own  family.  Those  who  object  to 
nomenclature  of  this  sort  will  prefer  the  term  Myotonia  congenita,  but  as 
Thomserr  was  also  a  subject  of  the  disease  an  exception  may  well  be  made. 
Very  few  cases  have  been  described  in  America,  and  the  only  one  which 
the  author  has  had  an  opportunity  of  seeing  was  the  one  presented  to  the 
New  York  Neurological  Society,  in  1886,  by  G.  W.  Jacoby.  The  best 
accounts  of  the  disease  are  those  furnished  by  Thomsen  himself,  a  descrip- 
tion of  it  in  Leyden's  work  on  "Spinal  Cord  Diseases,"  and  the  mono- 
graph of  Erb,  who  summarized  the  chief  points  of  twenty-two  cases,  and 
added  a  full  account  of  peculiar  electrical  changes  in  this  disease,  for 
which  he  proposed  the  term  "  Myotonic  Reaction. "  Dejerine  and  Sottas, 
Schiefferdecker  and  others  have  more  recently  contributed  to  the  pathology 
of  the  disease. 

The  chief  symptoms  of  the  disease  are  a  rigidity  of  the  muscles  (myoto- 
nic contraction),  which  is  developed  whenever  an  attempt  is  made  to  use 
a  muscle  or  muscles  after  a  period  of  rest.  If  the  first  difficulty  has  been 
overcome,  the  action  of  the  muscles  may  be  entirely  normal  for  some  period 
of  time.  This  muscular  rigidity  is  most  noticeable  in  the  attempt  to  rise 
after  a  person  has  been  quietly  seated  for  some  time  ;  in  the  attempt  to  use 
the  hands  in  lifting,  grasping,  or  writing,  and  at  times  considerable  difficulty 
is  experienced  in  the  movements  of  the  tongue  and  of  all  muscles  concerned 
in  articulation.  The  impression  created  by  a  patient  grasping  the  physician's 
hand  and  then  not  being  able  to  let  go,  or  by  his  falling  to  the  ground  and 
remaining  absolutely  rigid  until  the  spasm  is  relaxed,  is  one  not  easily  for- 
gotten. The  spasms  are  generally  limited  to  a  few  groups  of  muscles,  but  in 
some  cases  the  entire  muscular  system,  with  the  possible  exception  of  the 
ocular  and  respiratory  muscles,  is  involved.  The  rigidity  of  the  muscles  is  in- 
creased under  the  influence  of  emotional  excitement  and  under  the  influence 
of  cold  and  damp  weather.  Under  alcoholic  stimulants  the  rigidity  is  said  to 
lessen,  and  prolonged  periods  of  rest  undoubtedly  have  a  favorable  influence 
upon  the  disease. 

The  disease  occurs  in  families,  and  very  often  affects  several  members  of 
the  same  family.  In  Thomsen's  family,  five  generations  have  been  affected 
by  this  same  condition.  There  seems  to  be  no  distinction  as  regards  sex, 
and  the  disease  may  come  on  at  any  period  of  life.  A  number  have  been  re- 
corded during  the  first  decade  of  life,  but  the  disease  is  apt  to  reach  its  maxi- 
mum during  the  period  of  greatest  muscular  development,  and  the  symptoms 
are,  therefore,  pronounced  between  the  ages  of  fifteen  and  twenty-five.  In 
Jacoby's  case  the  patient,  who  was  twenty-four  years  of  age  at  the  time  of 


140  THE  NERVOUS  DISEASES   OF   CHILDREN. 

examination,  stated  distinctly  that  he  could  not,  as  a  boy,  take  part  in  the 
outdoor  games  of  his  comrades  on  account  of  the  stiffness  of  his  muscles. 
He  attempted  to  play  upon  the  organ,  but  found  that  his  hands  were  clumsy. 
He  was  not  able  to  whistle,  and  even  in  chewing  his  food  the  muscles  be- 
came stiff  and  rigid,  simulating  a  condition  of  trismus.  In  this  case  there 
was  distinct  rigidity  of  the  muscles  of  the  eyes. 

The  entire  muscular  system  is,  as  a  rule,  well  developed  ;  there  is  often  an 
increase  of  normal  volume.  According  to  Thomsen,  the  more  the  muscles 
are  used  the  less  the  spasm  becomes,  and  he  advises  a  very  active  life  as  pos- 
sibly the  only  hope  for  an  improvement  in  the  disease. 

There  are  a  few  objective  symptoms  which  render  the  diagnosis  of  the 
disease  easy  enough.  The  mechanical  excitability  of  the  muscles  is  mark- 
edly increased,  a  single  tap  of  the  hammer  on  a  muscle  produces  a  slow,  tonic 
contraction  of  the  fibres,  but  the  contraction  is  not  relaxed  for  some  period 
of  time.  In  contradistinction  to  the  symptoms  of  tetany  it  may  be  stated 
that  the  nerves  do  not  show  any  increased  mechanical  excitability.  There 
is,  furthermore,  a  very  remarkable  change  in  the  behavior  of  the  muscles  un- 
der electrical  stimulation.  Erb  has  proposed  to  speak  of  this  as  "  myotonic 
reaction."  The  faradic  excitability  of  the  nerves  is  not  changed,  but  on  the 
use  of  a  strong  current  the  muscles  innervated  by  the  nerve  will  be  forcibly 
contracted  and  remain  contracted  for  some  period  of  time  after  the  current  is 
broken.  The  direct  faradic  excitability  of  the  muscles  is  very  much  in- 
creased, mild  currents  being  sufficient  to  produce  contractions  of  long  dura- 
tion. The  galvanic  excitability  of  the  nerves  is  surely  not  increased — possi- 
bly diminished — but  the  same  muscular  phenomenon  can  be  observed  on 
galvanic  stimulation  of  the  nerve  as  in  the  case  of  faradic  excitation.  The 
direct  galvanic  excitability  of  the  muscles  is  increased ;  the  anodal  contrac- 
tion is,  as  a  rule,  greater  than  the  kathodal  contraction ;  opening  contrac- 
tions either  with  the  anode  or  kathode  cannot  be  readily  obtained.  The  con- 
tractions are  sluggish,  and  are  continued  for  some  time  after  the  elec- 
trical stimulation  ceases.  But  the  most  characteristic  symptom  of  this 
myotonic  reaction  is  a  peculiar,  rhythmical,  wave-like  contraction  which  pro- 
ceeds from  the  kathode  toward  the  anode.  This  phenomenon  can  best  be 
observed  with  the  use  of  strong  currents  of  at  least  20  or  25  milliamperes, 
and  if  the  negative  pole  is  placed  over  the  tendinous  end  of  a  muscle.  If 
the  kathode  is  placed  in  the  palm  of  the  hand,  the  anode  on  the  shoulder,  a 
wave-like  contraction  will  appear  and  gradually  work  its  way  from  the  mus- 
cles near  the  wrist  to  those  of  the  shoulder.  The  response  to  the  static  cur- 
rent remains  normal. 

These  are  the  chief  symptoms  of  the  disease.  A  few  complications  have 
been  observed  in  some  of  the  cases,  among  which  we  may  specially  mention 
increased  mental  irritability  and  hypochondriasis,  both  of  which  are  not  un- 
natural in  view  of  the  annoyance  which  the  disease  causes  the  sufferer. 
Epilepsy  and  migraine  have  also  been  recorded  in  connection  with  Thorn- 
sen's  disease,  but  as  both  these  diseases  are  to  a  great  degree  hereditary  the 
association  may  be  simply  a  coincidence. 

The  pathology  of  the  disease  is  still  obscure.     Dejerine  and  Sottas  found 


PA  RAMYOCL  ONUS  MULTIPLEX.  1 4 1 

no  changes  in  the  nervous  system,  but  endorse  the  findings  previously  made 
out  in  muscular  tissue  excised  from  the  living  body.  The  changes  noted 
were  an  enormous  hypertrophy  of  the  fibres,  with  considerable  increase  of 
the  nuclei  in  the  sarcolemma,  and  an  increase  of  the  interstitial  connective 
tissue.  In  one  case  examined  by  Erb,  there  was  a  striking  vacuolization  of 
the  individual  muscular  fibre.  These  histological  findings  do  not  enable  us 
as  yet  to  explain  the  character  of  the  disease.  Thomsen  considers  defective 
cortical  innervation  to  be  the  prime  cause,  but  cerebral  changes  alone  could 
not  account  for  all  the  symptoms ;  these  must  be  ascribed  to  disease  (pri- 
mary or  secondary)  of  the  muscles  or  to  disease  of  the  cord.  Some  have 
been  inclined  to  attribute  the  disease  to  auto-intoxication.  Proof  is  want- 
ing; the  findings  point  merely  to  an  abnormal  development  of  the  muscular 
system. 

The  disease  can  be  mistaken  only  for  tetany,  and  possibly  for  chronic  mus- 
cular dystrophies  ;  but  as  Erb  suggested,  several  years  ago,  a  tap  with  the 
percussion-hammer  and  a  few  closure  contractions  with  kathode  and  anode 
upon  certain  muscles  will  suffice  for  a  diagnosis  of  Thomsen's  disease. 

The  prognosis  is  favorable  as  regards  life,  unfavorable  in  regard  to  a  cure 
of  the  disease.  Much  can  unquestionably  be  done  by  a  continued  exercise  of 
the  will  and  by  regular  gymnastic  exercise.  No  further  treatment  of  the  dis- 
ease is  indicated. 

CONGENITAL   PARAMYOTONIA. 

Eulenburg  *  has  described  a  somewhat  similar  affection,  which  he  terms 
"  Congenital  Paramyotonia."  This  also  is  a  distinctly  family  disease  which 
may  be  traced  through  six  generations,  the  disease  becoming  manifest  im- 
mediately after  birth.  The  chief  feature  of  these  cases  is  a  tonic  spasm  last- 
ing from  a  quarter  of  an  hour  to  several  hours,  and  coming  on  chiefly  after 
exposure  to  cold.  There  is  distinct  weakness  associated  with  rigidity  ;  the 
facial  muscles  may  be  involved  as  well  as  the  orbicularis  palpebrarum  and 
the  orbicularis  oris,  making  speech  impossible  during  the  continuance  of  the 
contraction.  The  disease  is  differentiated  from  Thomsen's  disease  by  the 
absence  of  increased  mechanical  excitability  and  by  the  absence  of  anything 
resembling  a  myotonic  reaction.  Eulenburg  states  that  there  is  a  distinct 
tendency  to  kathodal  and  anodal  tetanus. 


PARAMYOCLONUS   MULTIPLEX   (MYOCLONIA). 

A  short  reference  is  necessary  in  this  connection  to  the  above  disease, 
which  bears  a  resemblance  to  electrical  chorea  and  to  tic  convulsif.  By 
some  it  has  been  regarded  as  a  form  of  hysteria,  but  this  is  not  warranted, 
for  cases  have  been  observed  in  which  there  were  none  of  the  characteristic 
signs  of  hysteria.  It  is  best  to  restrict  the  term  paramyoclonus  multiplex 
to  the  disease  as  it  was  originally  described  by  Friedreich.  The  chief  symp- 
*  Neurologisches  Centralblatt,  June  15,  1886. 


142  THE  NERVOUS  DISEASES   OF   CHILDREN. 

torn  of  the  condition  is  a  rapidly  repeated  clonic  spasm,  occurring  in  attacks 
and  affecting  individual  muscles  or  groups  or  muscles,  and  as  a  rule  symme- 
trical muscles  are  involved.  The  muscles  of  the  extremities  are  more  fre- 
quently affected  than  those  of  the  other  parts  ;  the  face  muscles  are  gener- 
ally exempt,  and  in  this  respect  the  disease  differs  markedly  from  other  forms 
of  choreic  and  clonic  spasm.  These  clonic  convulsions  are  not  severe 
enough  to  cause  actual  movements  of  the  extremities,  but  in  a  few  cases  sud- 
den contractions  of  the  diaphragm  have  been  accompanied  by  hiccough  and 
by  peculiar  respiratory  sounds,  and  in  the  case  described  by  Starr  the  move- 
ments were  strong  enough  to  produce  jactitation  of  the  head  and  of  the 
trunk.  In  contrast  to  some  of  the  other  diseases  described  in  this  chapter, 
the  myotatic  irritability  is  increased  only  a  little  or  not  at  all.  Electrical  ex- 
citability is  not  altered. 

The  disease  may  come  on  at  any  period  of  life  between  the  age  of  pu- 
berty and  fifty  years.  It  is  rare  in  young  children,  and  the  short  account  of  it 
here  is  simply  given  for  the  purpose  of  showing  its  close  relationship  to  the 
various  forms  of  choreic  disorders.  Heredity  is  not  a  prominent  factor  in 
the  cases  which  have  been  described,  but  it  has  been  found  in  several  mem- 
bers of  one  family.  Mental  or  physical  strain  and  emotional  excitement  are 
the  chief  causes.  Cases  closely  resembling,  yet  not  identical  with  paramyo- 
clonus multiplex  have  been  described  by  Hammond  and  others,  under  the 
title  of  "  Convulsive  Tremor." 

The  prognosis  is  serious  as  regards  a  cure  of  the  condition,  though  some 
of  the  cases  have  been  reported  improved  and  a  few  entirely  cured.  In  the 
treatment  of  the  disease  nothing  more  can  be  attempted  than  regular  gym- 
nastic exercise,  and  the  exhibition  of  mild  sedatives,  such  as  chloral  and  the 
bromides,  in  small  doses,  in  case  the  spasms  are  excessive.  A  mild  galvanic 
current  is  said  by  some  to  have  exercised  a  favorable  influence  over  the  disease. 

Dana  has  endeavored  to  establish  a  classification  of  myoclonias  approved 
by  Lundborg.  It  is  as  follows  :  i.  Myoclonia  of  Friedreich,  or  peripheral  type 
including  myokymia ;  2.  Myoclonia  of  the  functional  or  hysterical  type ; 
3.  Myoclonia  of  the  convulsive  tic  type  (many  associated  spasms,  choreic 
and  tonic)  ;  4.  Myoclonia  of  the  "  familial "  type  or  myoclonus  epilepsy — 
closely  associated  with  the  third ;  5.  Myoclonia  of  the  type  of  the  infectious 
and  symptomatic  choreas.     (Clark  and  Prout.) 


MYOCLONUS   EPILEPSY. 

Unverricht  has  described  a  form  of  myoclonus,  occurring  as  a  family 
disease  and  associated  with  epilepsy.  In  addition  to  the  general  clonic 
movements,  the  tongue,  the  muscles"  of  deglutition,  and  even  the  dia- 
phragm may  be  involved.  The  epileptic  seizures  may  be  few  and  far  be- 
tween in  earlier  years,  are  often  nocturnal ;  later  on  epileptic  attacks  occur 
more  frequently.  Lundborg,  Clark  and  Prout  have  given  especial  attention 
to  this  association  disease.  (See  Clark  and  Prout's  article,  American  Journal 
of  Insanity,  No.  2.     1902.) 


CHAPTER  VII. 

TETANUS. 

In  view  of  recent  bacteriological  researches  tetanus  may- 
be described  as  an  infectious  disease  of  the  central  nervous 
system,  characterized  by  continuous  tonic  spasms  with  oc- 
casional attacks  of  clonic  movements.  The  muscles  first 
affected  are,  as  a  rule,  those  of  the  neck  and  jaw.  Opistho- 
tonus and  lock-jaw  (trismus)  are  the  first  and  most  startling 
symptoms  of  the  disease.  According  to  its  origin  we  dis- 
tinguish between  traumatic  tetanus  resulting  from  a  wound, 
and  idiopathic  or  rheumatic  tetanus  if  the  cause  is  unknown, 
or  if,  in  our  ignorance,  we  attribute  it  to  exposure  to  wet 
and  cold.  We  must  take  special  note  of  the  tetanus  oc- 
curring in  new-born  children  (tetanus  neonatorum),  and 
may  make  a  passing  reference  to  puerperal  tetanus.  There 
is  every  reason  to  believe  that  the  tetanus  bacillus,  discov- 
ered by  Nicolaier  and  developed  in  pure  cultures  by  Kit- 
asato,  or  its  toxic  product  (studied  by  Brieger  chiefly)  is 
the  direct  cause  of  tetanus. 

Etiology. — Tetanus  is  far  more  frequent  in  males  than 
in  females.  This  is  particularly  true  of  those  cases  in  which 
the  traumatic  factor  is  evident,  and  is  easily  explained  by 
the  greater  liability  of  the  male  sex  to  traumatism.  The 
very  fact  that  of  46  so-called  idiopathic  cases  recorded  by 
Gowers  only  9  were  in  females  would  lead  one  to  doubt 
the  spontaneous  origin  of  these  cases.  While  tetanus  may 
occur  at  any  time  of  life,  it  is  most  frequent  between  the 
ages  of  ten  and  forty  years.  It  occurs  within  the  first  few 
days  of  life  between  the  fourth  and  eleventh  days,  but  be- 
tween this  time  and  the  age  of  five  years  there  is,  practically, 
immunity.  Between  five  and  ten  years  the  tendency  to 
the  disease  is  on  the  increase,  and  continues  to  increase  un- 
til after  the  age  of  forty  years.     These  statistics  strengthen 


144  THE  NERVOUS  DISEASES   OF   CHILDREN. 

the  impression  that  traumatic  lesion  alone,  plus  infec- 
tion, is  the  only  feasible  explanation  of  the  occurrence  of 
the  disease. 

Colored  races,  and  particularly  those  living  in  tropical 
countries,  are  affected  more  frequently  than  white  people. 
This  has  been  amply  proved  in  regard  to  the  population  of 
India  and  the  West  Indies.  The  filth  and  uncleanly  habits, 
so  common  among  these  people,  should  be  borne  in  mind. 
Puerperal  tetanus,  which  is  unquestionably  infective  in 
character,  occurs  very  frequently  still  in  these  same  coun- 
tries, but  it  is  becoming  less  and  less  frequent  in  those 
countries  in  which  modern  surgical  principles  prevail  in 
obstetrical  practice. 

The  previous  health  of  the  person  seems  to  have  little  to 
do  with  the  development  of  the  disease.  The  most  robust 
as  well  as  the  feeblest  are  equally  liable  to  the  infection. 
Irregular  wounds,  burns,  scratches,  and  frost-bites  are  more 
often  the  cause  of  tetanus  than  the  laige  aseptic  incisions 
made  by  the  surgeon's  knife.  Tetanus  has  been  known  to 
follow  the  extraction  of  a  tooth,  and,  in  the  popular  belief, 
lock-jaw  is  much  feared  after  slight  injuries  to  the  toes 
in  paring  nails,  etc.  With  greater  cleanliness  among  the 
masses  the  disease  will  no  doubt  soon  diminish.  Tham- 
hayn  states,  from  a  large  collection  of  cases,  that  tetanus 
is  developed  most  frequently  from  wounds  in  the  hand,  leg, 
foot,  head  and  neck,  arm  and  trunk,  in  the  order  of  fre- 
quency just  mentioned.  Injuries  to  the  head  have  often 
been  supposed  to  be  peculiarly  liable  to  produce  tetanus ; 
but  as  such  injuries  are  generally  the  result  of  a  fall  and 
contact  with  the  earth,  as  in  a  fall  from  a  horse,  it  will  be 
seen  that  the  danger  of  infection  is  greater  than  in  many 
other  instances.  The  same  is  true  of  tetanus  following  a 
splinter  which  has  been  run  under  the  nail,  and  the  old- 
fashioned  method  of  stopping  bleeding  by  putting  cobwebs 
around  the  bleeding  part  may  be  very  largely  responsible 
for  the  tetanus.  The  micro-organisms  of  tetanus  evidently 
do  not  require  a  large  abraded  surface,  and  it  is  question- 
able whether,  even  in  those  cases  in  which  tetanus  comes  on 
after  flogging,  there  was  not  sufficient  abrasion  of  the  epithe- 
lium to  permit  of  the  invasion  of  these  minute  organisms. 


TETANUS.  I45 

As  a  rule,  tetanus  is  developed  within  a  period  of  five  to 
fourteen  days  after  the  initial  injury;  but,  in  some  cases,  a 
few  hours  are  sufficient  for  the  development  of  the  disease, 
and  in  cases  in  which  the  crural  nerve  has  been  enclosed 
by  a  ligature,  full-fledged  tetanus  of  the  severest  type  was 
observed  at  once  after  the  occurrence.  The  development 
of  tetanus  is  not  rare  within  three  or  four  weeks  after  the 
injury,  but  beyond  such  a  period  the  occurrence  of  tetanus 
could  not  well  be  explained.  That  infection  alone  will  not 
explain  all  the  circumstances  in  connection  with  the  de- 
velopment of  tetanus  will  be  insisted  on  in  the  section  on 
pathology. 

Symptoms. — The  first  symptoms  of  tetanus,  if  we  except 
vague  pains  at  the  seat  of  injury,  and  a  dull  headache,  is 
a  gradual  stiffening  of  the  muscles  of  the  neck  and  of  the 
jaw  until  the  head  is  markedly  retracted  (opisthotonus) 
and  the  jaws  are  firmly  clenched  (trismus).  While  the  cer- 
vical muscles  are  apt  to  be  involved  at  the  beginning  the 
dorsal  and  lumbar  apparently  escape ;  but  retraction  of 
the  head  is  soon  followed  by  arching  of  the  back.  As  the 
disease  continues  the  legs  become  rigid,  but  the  arms  es- 
cape or  are  but  little  involved.  The  muscles  of  the  face 
are  often  affected  at  an  early  stage  of  the  disease.  The  eye- 
brows are  raised,  the  ocular  fissure  becomes  narrow,  the 
mouth  is  distorted,  the  lips  press  against  the  teeth  and  the 
entire  face  may  assume  what  is  known  as  the  "  risus  sar- 
donicus."  The  rigidity  of  the  muscles  is  painful,  and  the  oc- 
casional clonic  exacerbations  which  occur  tend  to  make  the 
condition  still  more  distressing.  The  difficulties  of  degluti- 
tion and  of  respiration,  from  spasm  of  the  thoracic  muscles 
and  of  the  glottis,  increase  the  agony  of  the  patient  and  are 
a  continuous  menace  to  life.  Epigastric  pains  (pains  possibly 
due  to  spasm  of  the  diaphragm)  are  frequently  complained 
of.  During  sleep  the  spasm  usually  ceases,  but  no  sooner 
does  the  patient  wake  up  than  all  the  distressing  symptoms 
return  in  full  force. 

The  pulse  is  rapid  and  feeble.  Whether  vaso-motor 
spasm  is  the  cause  of  this  peculiarity  of  the  pulse  has  not 
been  sufficiently  determined.  The  temperature  varies 
considerably  ;  in  some  instances  it  remains  normal  during 


I46  THE  NERVOUS  DISEASES   OF   CHILDREN. 

the  entire  course  of  the  disease,  in  others  there  is  a  con- 
tinuous fever  of  from  two  to  three  degrees.  H.  C.  Wood 
refers  to  the  serrated  tracings  of  the  temperature  chart, 
the  rise  coinciding  with  the  paroxysm,  and  the  fall  of  the 
line  with  the  interval.  Occasionally  the  temperature  rises 
to  1080  or  even  no0  F.,  and  according  to  Wunderlich  this 
high  temperature  continues  for  some  hours  after  death, 
reaching  even  to  1 140  F.  It  was  formerly  supposed  that  the 
rise  of  temperature  was  due  to  excessive  muscular  action ; 
but  cases  in  which  muscular  action  is  greatest  are  not  nec- 
essarily those  with  highest  or  even  high  temperature. 
Gowers  is  inclined  to  consider  the  fever  to  be  of  nervous 
origin,  which  is  probably  correct  if  modified  to  mean  that 
the  toxines  circulating  in  the  system  exercise  a  special  ir- 
ritating influence  upon  the  centres  in  the  pons  and  spinal 
cord,  or  upon  the  heat  areas  in  the  cortex.  Thirst  is  a  fre- 
quent and  most  distressing  symptom  of  the  disease,  and  as  it 
cannot  be  easily  quenched,  on  account  of  the  difficulty  of 
deglutition,  it  adds  much  to  the  discomfort  of  the  patient. 
The  thirst  is  also  increased  by  the  very  profuse  perspira- 
tion which  sets  in  and  is  a  prominent  factor  in  many  of  the 
cases. 

The  urine  is  scanty  and  high-colored,  of  high  specific 
gravity,  all  of  which  can  be  explained  by  the  excessive  ac- 
tion of  the  skin.  Micturition  is  often  irregular,  possibly  in 
consequence  of  spasm  of  the  sphincter.  The  bowels  are,  as 
a  rule,  constipated. 

All  the  symptoms  are  apt  to  increase  in  intensity  as  the 
disease  progresses.  The  majority  of  cases  run  to  a  fatal 
termination  in  less  than  a  fortnight,  often  in  four  to  five 
days.  Death  occurs,  as  a  rule,  either  from  failure  of  the 
heart  or  from  asphyxia  in  consequence  of  spasm  of  the  re- 
spiratory muscles.  In  other  cases  exhaustion  from  the  con- 
tinued spasms  and  from  the  inability  to  take  food  may  be 
the  ultimate  cause  of  death.  Cases  lasting  above  a  fortnight 
are  supposed  to  tend  toward  recovery.  The  spasms  be- 
come slighter,  the  clonic  spasm  less  frequent,  and  all  the 
symptoms  gradually  recede  until  nothing  but  a  slight  rigid- 
ity of  the  muscles  remains.  The  parts  last  involved  are  first 
released,  and  the  spasms  last  longest  in  the  muscles  of  the 


TETANUS.  I47 

neck  and  jaw,  the  very  parts  which  were  first  affected.  A 
single  recovery  seems  to  provide  immunity  against  the  dis- 
ease for  all  time.  While  the  evidence  upon  this  point  is 
not  absolutely  conclusive,  the  fact  of  immunity  after  the 
first  attack  would  be  in  keeping  with  what  we  know  about 
immunity  from  other  infectious  diseases. 

Varieties. — The  majority  of  the  cases  are  so  much  alike 
that  it  would  be  quite  useless  to  speak  of  variations  from 
the  clinical  type.  An  exception  must  be  made,  however, 
in  favor  of  what  Rose  has  termed  "  cephalic  tetanus."  This 
form  develops,  as  a  rule,  after  injury  to  the  head,  and  partic- 
ularly to  the  region  of  the  face  supplied  by  the  fifth  nerve. 
The  special  characteristics  of  this  form  are  the  association 
of  paralysis  of  the  face  on  the  same  side  as  the  injury  with 
tetanic  spasms  of  the  other  side.  In  addition  to  this  we 
find  spasms  of  the  respiratory  muscles  and  great  difficulty 
in  deglutition.  The  behavior  of  the  patient  is  very  much 
like  that  of  one  suffering  from  hydrophobia,  hence  this 
form  has  also  been  spoken  of  as  "  tetanus  hydrophobicus." 
There  is  some  doubt  whether  injury  to  the  peripheral  nerve 
is  the  cause  of  the  facial  palsy.  Bernhardt  and  Remak 
proved  conclusively  that  there  was  no  reaction  of  degen- 
eration in  the  nerve  or  muscles,  and  the  nerve  has  been 
found  healthy  on  post-mortem  examination.  We  must 
suppose,  therefore,  that  the  facial  palsy  in  such  cases  is  of 
reflex  origin.  Some  support  is  given  to  this  view  by  the 
fact  that  if  recovery  sets  in,  the  tetanic  spasm  and  the  facial 
palsy  disappear  at  about  the  same  time. 

The  only  other  variety  worthy  of  special  mention  is  the 
so-called  abortive  form  of  tetanus  described  by  Kussmaul. 
In  these  cases  spasm  of  the  neck  muscles  and  trismus  are 
the  only  symptoms,  and  these  gradually  disappear. 

Tetanus  neonatorum  can  hardly  be  considered  a  variety 
of  tetanus,  for  it  resembles  in  every  way  the  tetanus  of  the 
adult,  but  in  this  book  it  will  naturally  deserve  some  spe- 
cial consideration. 

The  tetanus  of  the  new-born  child  sets  in,  as  a  rule,  be- 
tween the  fifth  and  tenth  days  after  birth,  though  it  may  be 
delayed  as  long  as  twenty  days.  The  first  symptom  noticed 
in  the  child  is  difficulty  in  taking  the  nipple  or  in  drinking 


148  THE  NERVOUS  DISEASES   OF   CHILDREN. 

from  the  bottle ;  with  every  attempt  there  is  distinct  rigid- 
ity of  the  masseters  and  of  the  muscles  about  the  mouth, 
which  interferes  with  the  act  of  sucking  and  swallowing. 
Other  facial  muscles  are  apt  to  be  in  a  state  of  contracture 
leading  to  a  distinct  deformity  of  the  face.  In  some  in- 
stances food  can  be  poured  into  the  mouth  by  a  spoon  with- 
out exciting  a  spasm,  but  in  many  others  the  mere  touch  of 
the  spoon  to  the  lips,  or  contact  of  the  food  with  the  lips 
and  mouth,  is  sufficient  to  produce  a  spasm. 

In  the  earlier  stages  of  the  disease  the  child  is  quiet  un- 
less food  is  given;  but  before  long  the  spasms  come  on 
spontaneously  without  excitation  by  food.  Spasm  of  the 
respiratory  muscles  soon  forms  a  part  of  the  clinical  symp- 
toms, and  periodic  cyanosis  is  often  one  of  the  early  symp- 
toms of  the  tetanic  condition.  Rigidity  of  the  muscles  of 
the  neck  and  back,  typical  opisthotonus,  and  arched  back 
soon  follow,  and  to  make  the  symptoms  thoroughly  com- 
plete the  upper  and  lower  extremities  become  the  seat  of 
muscular  spasms.  There  may  be  slight  remissions  for  a 
time,  but  the  tetanic  stage  becomes  more  and  more  perma- 
nent, or,  if  absent  when  the  child  is  quiet,  will  surely  come 
on  with  the  merest  touch  in  lifting  the  child,  in  trying  to 
give  it  an  enema,  in  washing  it,  etc.  The  temperature,  as  a 
rule,  remains  normal ;  in  a  few  instances  it  reaches  1020  or 
103°  F. 

The  disease  is  steadily  progressive.  One  is  often  de- 
ceived by  slight  remissions  and  is  flattered  by  the  tempor- 
ary good  result  of  sedative  treatment,  but  is  only  too  often 
disappointed  by  the  reappearance  of  all  the  symptoms,  with 
even  greater  intensity.  Death  may  follow  within  a  period 
varying  from  one  and  a  half  to  eight  or  nine  days,  and  re- 
sults either  from  mere  exhaustion,  due  to  the  inability  to 
take  food,  or  from  asphyxia,  due  to  spasm  of  the  inspiratory 
muscles. 

A  few  cases  of  tetanus  neonatorum  get  well,  but  surely 
only  a  very  few.  Cases  with  fever  seem  to  be  invariably 
fatal.  In  those  who  get  well  the  tetanic  spasm  disappears 
very  slowly,  and  Henoch  states  that  in  some  instances  a 
rigidity  of  the  muscles  existed  three  weeks  after  the  onset 
of  the  disease. 


TETANUS. 


I49 


The  cause  of  this  early  tetanus  is  in  every  way  the 
same  as  that  of  tetanus  in  the  adult,  except  that  the  en- 
trance of  the  bacilli  into  the  body  occurs  by  way  of 
the  umbilical  cord,  the  care  of  which  has  been  neglected. 
In  former  days,  and  particularly  in  those  countries  in  which 
obstetrical  cases  were  managed  exclusively  by  midwives, 
whose  ideas  of  cleanliness  were  very  defective,  tetanus 
neonatorum  was  very  much  more  frequent  than  it  is  now. 
Ritual  circumcision,  if  performed  without  due  regard  to 
modern  surgical  principles,  may  be  considered  an  occasional 
cause  of  tetanus.  Bathing  a  child  at  low  temperatures  is 
referred  to  by  many  of  the  older  writers  as  a  common 
cause  of  tetanus.  The  use  of  a  bath  thermometer  is  said  to 
have  put  an  end  to  an  epidemic  of  tetanus  occurring  in  the 
practice  of  a  midwife  who  was  not  able  to  distinguish  by 
the  hand  alone  the  proper  temperature  of  water.  In  view  of 
the  modern  bacillary  theory  of  tetanus  such  an  origin  of  tet- 
anus would  be  rather  difficult  to  explain  ;  but  we  must  con- 
cede the  possibility  of  other  than  mere  toxic  agents  affecting 
and  irritating  the  peripheral  nerves  of  a  newly  born  child. 

Pathological  Anatomy  and  Morbid  Pathology. — 
Careful  search  has  been  made  by  many  writers  for  the  act- 
ual lesion  of  tetanus,  but  up  to  the  present  time  with  few 
positive  results.  It  has  been  noted  that  rigor  mortis  sets  in 
with  unusual  promptness,  some  claiming  that  the  tetanic 
spasm  passes  without  relaxation  into  the  post-mortem  con- 
tracture. All  the  organs  of  the  body  have  been  examined  ; 
no  special  changes  have  been  found  in  them.  Some  writers 
have  reported  oedema,  hypostatic  pneumonia,  pleural  ex- 
travasation, which  can  be  accounted  for  by  the  inter- 
ference with  the  circulation  and  respiration.  The  muscles 
of  the  body  are  sometimes  ruptured  ;  some  contain  small 
hemorrhages,  but  under  the  microscope  the  fibres  present 
no  anomalies,  though  Bowman  refers  to  occasional  granular 
degeneration.  It  has  been  natural  to  look  for  changes  in 
the  nerve  filaments  in  the  peripheral  wound  which  has  been 
the  starting-point  of  the  tetanus.  In  some  instances  noth- 
ing abnormal  has  been  found ;  in  others  there  has  been 
neuritis,  with  considerable  swelling  of  the  nerve,  which  ex- 
tended from  the  periphery  to  the  spinal  cord. 


i5o 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


In  the  case  of  tetanus  neonatorum  an  inflammation  of  the 
umbilical  vessels  has  been  traced  for  some  distance  within 
the  abdomen,  and  the  peritoneal  covering  of  the  vessels  has 
also  been  found  inflamed. 

As  for  the  central  nervous  system,  the  condition  most 
frequently  discovered  is  one  of  passive  distention  of  the 
vessels,  associated  with  minute  hemorrhages  ;  but  both  these 
conditions  are  surely  secondary  to  the  tetanic  spasms  and 
not  in  any  sense  the  cause  of  them.  In  the  spinal  cord  some 
investigators  have  reported  granular  disintegration  of  the 
gray  substance,  the  formation  of  cavities  containing  gran- 
ular material,  and  changes 
also  in  the  large  ganglion 
cells,  such  as  were  found  by 
Nerlich  in  the  motor  root 
of  the  fifth  nerve.  All  of 
these  changes  are  not  suffi- 
ciently distinct  to  represent 
the  actual  pathological  con- 
ditions of  tetanus  ;  they  are 
secondary  effects,  and  it  is 
probable  that  in  tetanus,  as 
in  other  of  the  diseases 
hitherto  considered,  definite 
though  the  symptoms  may 
be,  the  changes  are  of  a 
transitory  character,  every  trace  of  which  disappears  after 
death.  It  would  be  more  important,  therefore,  to  refer  to 
the  morbid  pathology. 

The  pathology  of  tetanus  can  be  readily  understood  in  the 
light  of  modern  researches  if  we  regard  the  spasms  of  tet- 
anus as  the  result  of  increased  irritability  of  the  convul- 
sive centres  in  the  brain.  This  increased  irritability  is  di- 
rectly due  to  the  influence  of  germs  or  germ  products 
introduced  into  the  system  from  some  external  injury. 
The  tetanus  bacillus  (Nicolaier)  is  a  rod-shaped  microbe 
whose  spores  are  attached  to  one  end  of  the  bacillus,  which 
with  its  spores  resembles  in  appearance  an  ordinary  pin. 
This  microbe  is  found  in  the  soil  as  well  as  in  the  dust  of 
dwellings.   These  bacilli  are  capable  of  resisting  great  heat, 


Fig.  49. — Tetanus  Bacilli  and  Spores, 
(x  about  1,000.)  (Kitasato.) 


TETANUS.  151 

retaining  their  activity  even  after  exposure  to  1750  F.  for 
an  hour  (Kitasato,  Zeitschrift  fiir  Hyg.,  vol.  vii.).  Their 
spores  are  capable  of  resisting  the  same  heat  for  six  hours, 
but  the  spores  are  not  formed  if  the  temperature  exceeds 
1080  F.  The  bacteriologic  phase  of  this  subject  has  been 
studied  most  carefully  in  recent  years  by  Ehrlich,  Wasser- 
man  and  Milchner,  to  whose  writings  the  reader  is  re- 
ferred. 

As  products  of  these  bacilli  we  have  several  poisons 
(tetanin,  tetano-toxin,  and  also  tox-albumins,  Brieger),  which 
have  been  derived  from  pure  cultures  and  which  are  ca- 
pable of  exciting  the  disease.  It  is  probable  that  the  tetanus 
bacillus  itself  does  not  cause  the  disease,  but  that  the  poi- 
sonous substances  formed  in  the  blood  by  the  presence  of 
this  bacillus  are  the  direct  cause  of  tetanus.  It  is  an  inter- 
esting fact  that  granulating  surfaces  do  not  offer  a  suitable 
soil  for  the  production  of  these  toxic  substances.  The 
presence  of  oxygen  seems  to  prevent  the  penetration  of  the 
bacilli  into  the  tissues  underneath ;  hence  we  can  readily 
understand  why  scratches  and  hidden  injuries  should  be 
far  more  dangerous  than  large  abraded  surfaces. 

Tetanus  was  supposed  to  result  from  direct  irritation  of 
the  peripheral  nerves ;  if  so,  an  ascending  neuritis  would 
have  to  account  for  the  development  of  the  symptoms  of 
tetanus.  It  is  at  present  altogether  impossible  to  deny  that 
in  some  way  or  other  the  bacillus  must  have  been  intro- 
duced into  the  system  even  in  these  cases.* 

Lesions  of  the  occiput  were  supposed  to  lead  more  fre- 
quently than  other  lesions  to  tetanus,  but  in  this  instance, 
too,  it  is  more  than  probable  that  the  infectious  element 
cannot  be  disregarded. 

In  no  disease  have  bacteriological  researches  led  to 
more  satisfactory  results  in  treatment.  Tizzoni  cultivated 
the  virus,  inoculations  of  which  in  increasing  strength  pro- 
duced tetanus  in  dogs.  The  blood-serum  of  such  dogs 
destroyed  the  activity  of  the  virus.     He  inferred  the  pres- 

*  Since  the  above  was  written  Goldscheider  has  given  a  different  explanation  based 
upon  recent  anatomical  conceptions.  The  poison  of  tetanus  acts  upon  the  peripheral 
nerve-fibre.  This  fibre  being  the  peripheral  portion  of  the  neuron,  an  increased  ex- 
citability of  the  ganglion  cell  is  produced,  and  this  hyper-excitability  brings  about 
the  tetanic  contractions  of  the  muscles  with  which  it  is  connected. 


152  THE  NERVOUS  DISEASES   OF  CHILDREN. 

ence  of  an  albuminoid  body,  which  he  called  "  anti-toxin," 
with  which  he  succeeded  in  arresting  the  disease  in  rats. 

The  now  famous  researches  of  Behring  and  Kitasato 
have  revealed  the  fact  that  the  blood-serum  of  tetanic  ani- 
mals produces  immunity  in  others,  and  that  the  serum  of 
the  animals  thus  rendered  immune  has  still  more  active 
anti-toxic  qualities.  It  was  during  the  year  1894  that  Beh- 
ring showed  that  the  blood-serum  of  immunized  animals  not 
only  produces  immunity  in  others,  but  also  has  a  distinctly 
curative  effect.  Tamu  ffi  (Centra/b/att  fiir  Bacteriologie,  1892) 
reported  a  successful  use  of  anti-toxin  injections  in  the  case 
of  a  peasant,  aged  seventy -four,  who  had  developed  tetanus 
as  the  result  of  a  lacerated  wound  of  the  little  finger  of  the 
right  hand.  One  hundred  and  fifty  grammes  of  the  anti- 
toxine  was  given  in  this  case,  when  on  the  eleventh  day 
after  the  beginning  of  the  injections  a  complete  recovery 
ensued.  The  blood-serum  from  this  patient  did  not  produce 
tetanus  when  injected  into  a  rat.  No  doubt  other  success- 
ful cases  of  this  kind  will  soon  be  reported,  and  there  is 
much  to  hope  from  this  serum  therapy  in  the  case  of  tet- 
anus. 

Differential  Diagnosis. — The  diagnosis  of  tetanus  is 
easy  in  the  majority  of  cases.  It  is  possible  to  mistake 
tetanus  for  strychnia  poisoning,  but  in  the  latter  the  symp- 
toms never  begin  with  distinct  trismus.  The  symptoms 
also  develop  with  much  greater  rapidity,  and  the  severe 
pain  referred  to  the  stomach,  as  well  as  the  absence  of  ex- 
ternal injury,  would  lead  one  to  suspect  the  effect  of  strych- 
nia. 

If  there  is  great  difficulty  in  deglutition  the  symptoms 
of  tetanus  may  resemble  somewhat  those  of  hydrophobia, 
but  the  entire  absence  of  trismus,  and  the  fact  that  all  the 
symptoms  are  excited  only  if  an  attempt  is  made  to  swallow, 
and  the  previous  history  of  the  case,  will  enable  one  to  dis- 
tinguish hydrophobia  from  true  tetanus. 

As  for  mistaking  tetanus  for  tetany,  such  confusion  is 
scarcely  possible  if  the  mode  of  onset  from  the  periphery 
inward,  in  cases  of  tetany,  is  kept  in  mind,  and  if  we  also 
remember  that  no  such  interval  exists  in  tetanus  as  in  the 
lesser  disease.     The  favorable  course  of  the   disease  will 


TETANUS.  153 

render  still  further  aid,  after  the  lapse  of  a  few  days,  in  dif- 
ferentiating between  tetany  and  tetanus. 

Prognosis. — The  prognosis  is  invariably  grave.  A  mor- 
tality of  eighty -five  per  cent,  does  not  overstate  the  facts. 
Other  things  being  equal,  the  prognosis  is  the  more  grave 
the  more  thoroughly  a  lacerated  wound  has  been  infected. 
Recovery  in  a  case  of  tetanus  after  compound  fracture 
of  the  limbs  is  very  rare.  The  longer  the  interval  between 
the  injury  and  the  first  onset  of  the  symptoms  the  better 
the  prognosis,  and  cases  which  begin  after  ten  days  usually 
take  a  more  favorable  course  than  those  which  come  on 
very  early  after  the  injury.  If  the  symptoms  have  lasted 
more  than  ten  days  there  is  some  reason  to  expect  a  fa- 
vorable result.  On  the  whole  it  is  a  disease  very  much  to 
be  feared,  and  a  guarded  prognosis  is  quite  in  order  until 
a  very  decided  improvement  sets  in  ;  but  possibly  the  recent 
advances  in  serum  therapy  may  in  the  course  of  time  en- 
able us  to  be  more  hopeful. 

Treatment. — In  the  treatment  of  tetanus,  prophylactic 
measures  are  by  far  the  most  important.  There  is  no  good 
excuse  at  the  present  day  for  the  infection  of  any  surgical 
wound,  or  if  the  wound  has  been  received  under  conditions 
which  make  infection  possible,  thorough  antiseptic  measures, 
should  be  at  once  employed,  and  even  after  the  first  symp- 
toms of  tetanus  have  been  observed  the  condition  of  the 
peripheral  wound  should  be  carefully  examined  into  and 
everything  should  be  done  to  prevent  further  contamina- 
tion and  infection. 

If  recent  investigations  are  carried  to  a  successful  issue, 
the  most  satisfactory  and  the  most  rational  treatment  of 
tetanus  will  unquestionably  be  that  by  injections  of  anti- 
toxin or  any  of  the  substances  which  may  hereafter  be 
proved  to  possess  the  property  of  counteracting  the  poison 
of  tetanus  in  the  human  system.  Behring  has  perfected  the 
anti-toxin  treatment  of  tetanus,  insisting,  also,  that  the  injec- 
tion be  made  as  near  as  possible  to  the  seat  of  infection. 

In  the  majority  of  cases,  and  particularly  in  those  occur- 
ring at  a  distance  from  large  medical  centres,  from  which  for 
years  to  come  these  anti-toxins  will  have  to  be  obtained,  the 
older  methods  of  treatment  will  still  have  to  be  resorted  to. 


154  THE  NERVOUS  DISEASES   OF   CHILDREN. 

The  general  management  of  the  patient  is  the  first  im- 
portant consideration.  Absolute  quiet  should  be  secured 
by  darkening  the  room,  keeping  out  all  visitors  and  noises. 
Nutritious  but  liquid  diet  should  be  given,  and  given  slowly, 
so  that  the  difficulties  in  deglutition  may  not  be  increased. 
If  the  trismus  is  very  marked  a  wedge  may  have  to  be  in- 
serted between  the  teeth  to  secure  enough  space  to  intro- 
duce a  tube  through  which  liquid  can  be  taken  into  the 
mouth.  If  this  cannot  be  done,  the  patient  must  be  fed 
through  the  nose  or  else  by  the  rectum. 

In  former  times  it  was  considered  wise  to  remove  a 
peripheral  scar,  or  even  to  amputate  the  part  that  included 
the  seat  of  injury  ;  but  according  to  our  present  conception 
the  presence  of  tetanus  bacilli  in  the  system  would  prove 
that  the  disease  was  no  longer  a  local  one,  and  that  excision 
of  the  lesion  would  not  prove  sufficient  to  bring  about  re- 
covery. 

Among  the  drugs  employed  in  the  treatment  of  this  dis- 
ease chloroform  by  inhalation  is  unquestionably  the  most 
satisfactory.  Woods,  of  Philadelphia,  has  recommended  the 
use  of  nitrite  of  amyl,  and  Gowers  reports  that  its  use  at 
Guy's  Hospital  has  proved  that  the  spasms  became  more  in- 
tense at  first  but  slighter  afterward.  Both  chloroform  and 
nitrite  of  amyl  are  palliative  measures,  but  not  curative  in 
any  true  sense.  Chloral  hydrate  has  an  advantage  over 
the  preceding  drugs,  inasmuch  as  it  helps  to  produce  sleep, 
and  can  be  given  continuously  for  a  long  period  of  time. 
To  be  effectual  large  doses  of  five  to  fifteen  grains  should  be 
given  at  a  time,  and  repeated  several  times  during  the  day 
according  to  the  needs  of  the  case. 

There  can  be  no  reasonable  objection  to  the  use  of  opium 
and  morphia  in  the  form  of  suppositories,  or  by  hypo- 
dermatic injection.  Morphia  may  be  exhibited  in  doses 
of  one-twelfth  to  one-sixth  grain,  according  to  the  age  of 
the  child,  and  can  be  given  in  connection  with  other  nar- 
cotics. The  use  of  conium  and  of  gelsemium  has  been 
warmly  recommended  by  various  observers,  but  the  results 
are  not  satisfactory  enough  to  advise  the  general  adoption 
of  such  measures.  Curara  in  doses  of  one-fiftieth  to  one- 
half  grain  every  hour  has  been  urged  by  some  ;  but  it  is 


TETANUS.  155 

surely  a  dangerous  drug,  and  its  application  would  be 
rather  in  the  nature  of  an  experiment  than  of  safe  thera- 
peusis.  Continued  warm  baths,  electricity  (galvanism  to 
the  spine  and  the  muscles),  all  these  measures  have  been  ad- 
vocated by  some,  but  without  much  result.  If  the  bromides, 
chloral,  opium  or  morphia,  and  chloroform,  judiciously  ad- 
ministered, do  not  bring  about  a  favorable  result  it  is  safer 
not  to  exhibit  other  drugs. 

In  the  treatment  of  tetanus  neonatorum  the  same  drugs 
may  be  employed  as  are  recommended  in  tetanus  of  the 
adult,  but  the  dosage  should  be  modified  in  keeping  with 
the  age  of  the  patient. 


CHAPTER  VIII. 

TETANY. 

Tetany  or  tetanilla  is  a  disease  which  occurs  with  far 
greater  frequency  in  Europe  than  in  this  country.  Osier 
and  Weir  Mitchell  speak  of  its  exceeding  rarity  in  the  United 
States.  It  occurs  with  about  equal  frequency  in  adults  and 
children.  As  the  name  indicates,  "  the  little  tetanus  "  is 
characterized  by  attacks  of  tonic  spasms  of  various  groups 
of  muscles,  particularly  of  those  of  the  upper  extremities. 
It  is  only  within  recent  years  that  the  disease  has  been  prop- 
erly studied,  though  the  first  account  of  it  goes  back  as 
far  as  the  year  1830,  when  Steinheim  described  this  group 
of  symptoms  as  a  special  form  of  articular  rheumatism.  In 
the  following  year  Dance  published  an  article  in  which  he 
expressed  the  view  that  the  intermittent  character  of  the 
spasms  proved  the  disease  to  be  of  a  malarial  order.  The 
name  of  the  disease  we  owe  to  Corvisart,  who  in  1852  re- 
viewed the  entire  subject.  His  observations  were  preceded, 
however,  by  those  of  Trousseau,  who  had  observed  the 
disease  in  nursing  women,  and  assumed  a  connection  be- 
tween tetany  and  the  function  of  lactation.  He  therefore 
termed  it  "  contracture  rheumatismal  des  nourrices,"  but 
later  on  he  observed  the  trouble  in  children  and  in  adults 
with  intestinal  obstruction,  and  so  abandoned  the  lactation 
theory.  It  was  Trousseau  who  first  discovered  the  very 
important  fact  that  these  attacks  could  be  excited  by  com- 
pression of  the  arteries  and  nerve-trunks  of  the  affected  ex- 
tremity. 

Our  knowledge  of  this  disease  has  been  enhanced  by  the 
observations  of  Chvostek,  Koppe,  Baginsky,  Von  Jaksch, 
Bernhardt,  and  Escherich.  The  most  comprehensive  articles 
on  the  subject  have  been  written  by  Weiss,  of  Vienna,  in 
1881,  by  Frankl  Hochwart  and  by  Escherich. 


TETANY. 


'57 


Symptomatology. — The  symptomatology  of  tetany  in- 
cludes the  symptoms  to  be  noticed  during  the  attack  and 
those  during  the  period  of  latency.     The  attack  is  preceded 


26>< 


Fig.  50.— Position  of  Hands  in  the  Spasm  of  Tetany.     (After  Oppenheim.) 

by  vague  tingling  pains,  by  formication  in  the  hands,  fore- 
arms,  and  legs;  a  feeling  of  stiffness  soon  follows,  and  subse- 
quently the  spasm  of  the  muscles  sets  in.     This  tonic  spasm 


158  THE   NERVOUS  DISEASES   OF   CHILDREN. 

occurs  more  frequently  in  the  upper  extremities,  and  gives 
rise  to  such  a  marked  rigidity  of  the  muscles  that  passive 
movements  are  for  the  time  being  impossible.  The  position 
of  the  hand  during  the  spasm  will  necessarily  vary  accord- 
ing to  the  groups  of  muscles  affected.  It  is  a  common 
occurrence  for  the  hand  to  assume  the  shape  of  the 
accoucheur's  hand.  Occasionally  also  the  thumb  is  very 
firmly  pressed  upon  by  the  flexed  fingers  and  the  nails  are 
buried  in  the  skin  of  the  palm.  In  some  rare  cases  there  is 
complete  extension  of  all  the  fingers.  The  forearms  are  gen- 
erally flexed,  and  the  upper  arms  closely  pressed  against  the 
chest.  If  the  lower  extremities  are  involved  the  thighs  may 
be  adducted,  the  legs  extended  or  flexed,  while  the  toes  are 
apt  to  assume  the  position  of  talipes  equinus.  The  spasms 
may  also  affect  the  muscles  of  the  abdomen,  of  the  chest, 
and  of  the  back.  These  tonic  contractions  of  the  abdomi- 
nal and  thoracic  muscles  may  interfere  with  the  movements 
of  the  diaphragm,  and  with  the  respiration,  causing  dyspnoea 
and  cyanosis.  If  the  muscles  of  the  neck  be  also  involved, 
the  return  of  the  venous  blood  from  the  brain  may  be  re- 
tarded, as  reported  by  Weiss  in  one  case,  in  which  loss  of 
consciousness  was  the  result  of  such  neck  spasm.  Opistho- 
tonus is  not  infrequent,  but  trismus  is  rare  and  never  occurs 
in  the  beginning  of  an  attack  as  in  tetanus.  In  severe  cases 
spasms  of  the  ocular  muscles  have  been  observed,  of  the 
oesophagus,  of  the  larynx,  and  of  the  muscular  apparatus  of 
the  bladder  (a  desire  to  urinate  but  micturition  impossible). 
During  the  attack  the  patient  complains  of  severe  pain  in 
the  affected  muscles ;  there  is  also  a  diminution  of  tactile 
sensibility  in  the  extremities,  the  muscular  sense  is  often 
deficient,  and  while  standing  on  the  floor  patients  have  a 
feeling  as  though  they  were  walking  on  velvet. 

Elevation  of  temperature  as  high  as  1040  F.  has  been  ob- 
served ;  but  this  is  exceptional,  as  Weiss  records  an  average 
of  only  one  such  case  in  twelve. 

Headache,  vertigo,  tinnitus  aurium,  and  excessive  perspi- 
ration are  other  symptoms  which  have  been  noted  during 
an  attack  of  tetany.  The  attacks  may  last  only  a  few 
minutes,  or  may  cover  a  period  of  hours,  or  even  days.  Se- 
vere attacks  of  tetany  may  bear  a  striking  resemblance  to 


TETANY.  I  59 

genuine  tetanus;  but  it  should  be  noted  that  there  is  no  ini- 
tial spasm  of  the  masseters  in  tetany,  and  that  in  this  form 
the  spasm  spreads  from  the  periphery  inward,  and  not  cen- 
trifugally,  as  is  the  case  in  tetanus.  Moreover,  the  reflex 
excitability,  is  not  nearly  as  great  in  tetany  as  in  tetanus. 
In  tetany  the  patients  may  be  entirely  free  from  attacks  for 
hours,  and  even  for  days,  whereas  in  tetanus  the  symptoms, 
as  long  as  the  attack  lasts,  are  continuous. 

Etiology. — The  disease  occurs  chiefly  in  very  young 
persons.  The  cases  observed  by  Koppe  were  all  from  one 
to  two  years  of  age.  Ganghofner  reported  40  cases  ;  of 
these  5  were  between  two  and  three  years  of  age  and  the 
remaining  35  between  the  ages  of  one  month  and  two  years. 
Gowers  tabulated  142  cases ;  of  these  64  occurred  from  one 
to  four  years,  and  36  from  ten  to  nineteen  years  of  age.  On 
going  over  the  literature  of  the  subject  a  few  years  ago,  taking 
all  cases  of  tetany  into  consideration,  it  was  evident  that 
the  majority  of  cases  of  tetany  were  observed  between  the 
ages  of  sixteen  and  thirty-five  years.  At  one  time  the  disease 
was  classed  among  the  professional  neuroses,  but  this  fallacy 
was  corrected  by  Kussmaul.  Any  exhausting  disease  may 
be  regarded  as  a  possible  etiological  factor,  but  exposure  to 
cold  and  wet  is  referred  to  most  frequently  as  a  predispos- 
ing cause.  Intestinal  irritation  is  another  cause,  and  may 
be  associated  with  eclampsia  and  laryngospasmus,  two  con- 
ditions which  we  know  are  also  frequently  excited  by  in- 
testinal irritation.  Riegel  instances  a  case  in  which  attacks 
of  tetany  were  inhibited  by  the  removal  of  the  ova  of  taenia 
medio-canellata  and  tricocephalus  dispar.  Weiss  reports 
the  occurrence  of  tetany  as  a  complication  of  typhoid  fever. 
It  has  also  been  observed  together  with  small-pox,  Bright's 
disease,  malaria,  chorea,  and  even  after  severe  mental 
shock.  The  only  just  inference  from  all  this  is  that 
tetany  is  liable  to  occur  after  any  exhausting  disease  in 
those  who  are  predisposed  to  this  form  of  spasm.  Weiss 
deserves  special  credit  for  bringing  into  prominence  the  re- 
lation between  tetany  and  extirpation  of  the  thyroid  gland. 
The  disease  sometimes  appears  in  epidemic  form,  if  we  are 
to  credit  the  account  of  such  occurrences  in  the  schools  and 
prisons  of  France.     A  similar  epidemic  occurrence  is  re- 


l6o  THE  NERVOUS  DISEASES   OF   CHILDREN. 

ported  by  J.  Lewis  Smith,  Escherich,  and  others.  That  it 
is  much  more  frequent  in  some  countries  than  in  others  has 
already  been  alluded  to. 

The  male  sex  is  affected  a  little  more  frequently  than  the 
female ;  Rilliet  and  Barthez  recording  20  out  of  28  cases  in 
boys.  In  Gowers's  statistics  of  142  cases,  76  were  males  and 
66  females.  Although  the  disease  sometimes  occurs  in 
families  there  seems  to  me  to  be  insufficient  evidence  of 
actual  hereditary  predisposition  to  this  disease. 

Symptoms  of  the  Latent  Period. — In  the  interval 
between  the  attacks  the  patient  may  appear  to  be  entirely 
well,  but  if  examined  carefully  he  will  exhibit  a  weakness, 
with  slight  rigidity  of  the  affected  muscles.  The  calf  mus- 
cles are  commonly  the  seat  of  mild  contractions.  Chvostek 
observed  slight  contractions  of  the  orbicularis  palpe- 
brarum. 

The  intervals  between  the  attacks  of  tetany  may  vary 
in  duration  from  several  hours  to  a  few  days,  a'nd  even  a 
few  months  ;  but,  of  course,  we  can  speak  of  a  latent  inter- 
val only  in  case  the  disease  can  be  proved  still  to  exist. 
This  can  be  done  by  eliciting  Trousseau's  symptoms  and 
by  proving  an  increased  electrical  and  mechanical  excita- 
bility of  the  parts  affected. 

Trousseau's  Symptom. — The  famous  French  physician 
discovered  that  in  persons  afflicted  with  tetany  a  charac- 
teristic attack  can  be  elicited  by  pressure  upon  the  large 
nerve-trunks  and  the  arteries  of  the  extremities  usually 
affected  during  an  attack.  The  attack  ceases  as  soon  as 
pressure  is  removed.  Kussmaul  and  Quincke  maintain 
that  in  some  cases  pressure  on  arteries  only  is  necessary, 
while  in  other  cases  the  slightest  pressure  on  a  nerve-trunk 
is  sufficient  to  produce  contractions  of  all  the  muscles  sup- 
plied by  this  nerve.  Trousseau's  symptom  is  absolutely 
pathognomonic  of  this  disease. 

Increased  electrical  excitability  has  been  found  to  be 
characteristic  of  tetany  by  Erb,  Chvostek,  Weiss,  and  oth- 
ers. According  to  these  authors  the  faradic  and  galvanic 
responses  of  the  motor  nerves  are  enormously  increased  dur- 
ing the  interval  between  the  attacks  of  tetany.  They  were 
not  only  able  to  obtain  kathodal  closure  contractions  (K. 


TETANY.  l6l 

C.  C.)  with  very  small  currents,  but  were  able  with  moder- 
ate currents  to  obtain  a  kathodal  closure  tetanus,  and  even 
an  anodal  opening  tetanus,  which  has  not  been  observed  in 
any  other  condition.  Chvostek  and  Weiss  claim  that  this 
phenomenon  is  exhibited  in  the  facial  and  in  other  periph- 
eral nerves.  Erb  found  the  electrical  excitability  greatest 
at  the  time  when  the  attacks  were  most  frequent. 

Increased  mechanical  excitability  is  a  still  more  striking 
symptom  of  the  condition,  a  simple  touch  with  a  percus- 
sion t  hammer  upon  a  nerve-trunk  being  sufficient  to  pro- 
duce contractions  of  the  muscles  supplied  by  the  nerve.  I 
remember  distinctly  the  cases  in  the  Vienna  General  Hospi- 
tal in  which  pressure  with  a  lead-pencil  upon  the  focal  point 
of  the  pes  anserinus  was  followed  by  contractions  exactly 
like  those  which  a  strong  faradic  current  applied  to  this 
part  would  have  produced.  This  is  by  far  the  most  con- 
venient test  to  make'in  cases  in  which  the  existence  of  tetany 
is  suspected,  and  it  is  far  better  to  endeavor  to  establish 
this  fact  of  increased  mechanical  excitability  than  to  excite 
an  attack  by  pressure  upon  a  large  nerve-trunk  or  a  large 
artery. 

Differential  Diagnosis. — There  can  be  but  little 
difficulty  in  differentiating  between  tetany  and  other  con- 
vulsive disorders.  From  tetanus  it  can  be  distinguished 
sufficiently  by  the  intermittent  and  centripetal  character 
of  the  attacks,  by  the  absence  of  trismus  at  the  beginning 
of  the  attack,  and,  above  all,  by  the  presence,  during  the  in- 
terval, of  Trousseau's  symptom,  and  the  increased  mechan- 
ical and  electrical  excitability.  I  have  known  a  case  of 
frequently  repeated  epileptiform  convulsions  to  be  mis- 
taken for  a  case  of  tetany,  but  the  loss  of  consciousness,  the 
universal  tonic  and  clonic  movements,  the  turning  of  the 
eyes,  the  stupor  following  an  attack,  and  the  extreme  short- 
ness of  the  attack,  are  the  symptoms  that  will  help  to  dif- 
ferentiate the  epileptiform  attack  from  one  of  tetany. 

Morbid  Anatomy  and  Pathology. — Post-mortem  ex- 
aminations of  cases  of  tetany  have  been  made  by  Langhans 
and  Weiss,  but  up  to  the  present  day  there  have  been  few 
positive  pathological  findings.  Langhans  claims  to  have 
found  a  periarteritis  and  periphlebitis  of  the  white  commis- 


1 62  THE  NERVOUS  DISEASES   OF  CHILDREN. 

sure  and  of  the  anterior  horns  of  the  cervical  portion  of  the 
spinal  cord.  Weiss  failed  to  find  any  such  changes  in  his 
cases.  He  formulated  an  ingenious  theory  of  the  disease 
according  to  which  the  attacks  of  tetany  are  due  to  an  irri- 
table condition  of  the  gray  matter  of  the  medulla  and  spinal 
cord.  This  irritable  condition  is  the  result  of  sympathetic 
disturbances  causing  irregularities  in  the  vascular  innerva- 
tion of  the  blood-vessels  of  the  spinal  cord.  Gowers  is  in- 
clined to  look  for  the  chief  changes  in  the  motor  cells  of 
the  spinal  cord,  but  all  this  is  in  the  nature  of  speculative 
pathology.  Schlesinger  has  very  recently  reviewed  the 
various  theories  concerning  the  nature  of  tetany.  He  con- 
cludes that  none  of  them  is  entirely  satisfactory,  while,  ac- 
cording to  his  own  ideas,  tetany  is  a  disease  of  the  entire 
nervous  system,  some  of  the  symptoms  being  due  to  an  af- 
fection of  the  peripheral  nerves  ;  but  the  tetanic  spasms, 
as  well  as  Trousseau's  phenomena,  are  held  to  be  the  result 
of  increased  excitability  of  the  gray  matter  of  the  central 
nervous  system,  chiefly  of  the  brain,  medulla  oblongata,  and 
spinal  cord.  Peripheral  irritants  of  various  kinds  may  give 
rise  to  vasomotor  disturbances  in  the  spinal  cord,  and  these 
may  be  the  cause  of  functional  changes.  This  last  theory 
is  but  a  little  more  satisfactory  than  the  preceding  ones,  for 
nothing  is  known  as  yet  of  that  special  peculiarity  and 
special  irritability  of  the  central  nervous  system  which 
give  rise  to  this  disease.  Irritability  of  the  nervous  system 
is  so  common  that  something  more  is  needed  to  explain 
these  very  unusual  manifestations  of  the  disease.  That  a 
zymotic  factor  enters  into  the  etiology  of  tetany  can 
scarcely  be  doubted,  but  the  exact  nature  of  such  infection 
is  a  matter  for  future  study. 

The  occurrence  of  tetany  after  extirpation  of  the  thyroid 
gland  points  the  way  to  a  future  investigation  regarding  the 
origin  of  the  disease.  If  it  is  the  function  of  the  thyroid 
gland  to  eliminate  mucin  from  the  body,  it  is  natural  to 
infer  that  the  continuance  of  this  toxic  substance  in  the  sys- 
tem is  responsible  for  the  tetanic  spasm  that  sets  in  when 
the  gland  is  removed  by  surgical  interference. 

A  special  caution  is  in  order  not  to  confound  ordinary 
carpopedal   spasm,  or  any  of   the  short  clonic  and  tonic 


TETANY.  163 

spasms  that  so  frequently  occur  in  children,  with  true 
tetany.*  The  term  should  be  restricted  to  those  cases  that 
present  distinct  attacks  and  a  free  interval,  characterized 
by  Trousseau's  symptom  and  increased  excitability. 

Prognosis. — The  prognosis  is  favorable  excepting  in 
those  few  cases  in  which  serious  lung  trouble  may  result 
from  continued  spasm  of  the  respiratory  muscles ;  but  the 
prognosis  should  be  very  guarded  with  respect  to  the  dura- 
tion of  the  disease.  It  may  vary  frOm  a  few  weeks  to  many 
months. 

Treatment. — The  removal  of  every  possible  peripheral 
irritation  is  the  first  sine  qua  non  of  treatment.  If  intestinal 
irritation  is  suspected  to  be  the  cause,  free  purging  of  the 
bowels  and  removal  of  intestinal  parasites  are  called  for.  A 
change  of  abode  may  become  necessary,  and  absolute  rest 
will  have  to  be  secured  at  any  cost. 

To  shorten  the  attack  the  physician  will  probably  have  to 
resort  to  the  hypodermic  use  of  morphine,  and  possibly  to 
hyoscine.  Weiss  reports  a  single  case  in  which  the  attack 
was  inhibited  by  the  application  of  ice  to  the  back  of  the  neck. 
Go  wers,  who  seems  to  think  the  spasms  of  tetany  very  closely 
related  to  ordinary  infantile  convulsions,  advises  treatment 
similar  to  that  employed  in  convulsions.  Proceding  on 
this  basis,  inhalations  of  nitrite  of  amyl  and  of  chloroform 
may  be  tried.  As  soon  as  the  attack  is  over  it  would  be 
well  to  administer  chloral  hydrate  in  daily  doses  of  about 
one-half  drachm,  or  the  bromides  in  doses  of  from  ten  to 
twenty  or  more  grains  per  day,  according  to  the  age  of 
the  child.  Small  doses  of  sulfonal,  or  of  trional,  are  worthy 
of  trial.  During  the  interval  careful  electrical  treatment 
(stabile  ascending  currents  through  the  peripheral  nerve- 
trunks)  as  well  as  prolonged  lukewarm  baths  should  be 
given.  There  is  little  doubt  but  that  the  majority  of  cases 
will  get  well  without  any  therapeutic  measures.  The  chief 
duty  that  devolves  upon  the  physician  is  clearly  that  of  in- 
vigorating the  central  nervous  system  by  the  best  known 
methods  and  remedies,  in  order  to  enable  it  to  resist  the 
ordinary  irritants  which  produce  the  disease  in  those  who 
are  predisposed  to  it.     If  the  child  exhibits  any  symptoms 

*  Some  of  the  cases  reported  by  Vaughan  are  not  distinct  cases  of  tetany. 


164  THE  NERVOUS  DISEASES   OF   CHILDREN. 

of  rickets,  cod-liver  oil  and  iron  will  be  the  best  remedies, 
and  treatment,  to  be  successful,  will  have  to  be  directed 
entirely  to  the  improvement  of  the  child's  defective  bone 
development.  The  bromides  may  be  administered  if  the 
condition  is  not  intense  enough  to  call  for  narcotics  and 
opiates,  and  if,  for  some  reason  or  other,  these  latter  reme- 
dies should  not  be  employed.  Gowers  states  that  a  dose  of 
digitalis,  given  at  bedtime,  has  been  found  to  be  the  most 
useful  remedy  for  nocturnal  tetany.  The  action  of  the  digi- 
talis could  be  explained  only  on  the  theory  that  the  blood- 
supply  of  the  central  nervous  system  is  deficient,  and  that 
there  is  need  of  increased  activity. 


TETANOID    CHOREA   AND    OTHER   TETANOID   DISORDERS. 

Under  this  title  Gowers  refers  to  a  case  which  has  been  under  his  care 
that  exhibited  symptoms  intermediate  between  those  of  chorea  and  that  of 
tetany.  The  disease  ran  a  fatal  course,  but  no  demonstrable  lesions  were 
found  after  death.  The  patient  was  a  boy,  ten  years  of  age,  with  a  history 
of  three  other  relatives  having  suffered  from  diseases  resembling  chorea.  In 
the  patient  the  symptoms  began  seven  months  before  death.  They  consisted 
of  tonic  spasm,  which  was  continuous,  and  varied  by  paroxysmal  attacks  of 
similar  but  more  intense  spa'sm.  The  face  was  involved  on  both  sides  so  as  to 
cause  a  constant  peculiar  smile.  The  tongue  was  pressed  up  against  the  pal- 
ate, impeding  swallowing  and  preventing  speech.  The  arms  were  extended, 
pronated,  and  rotated  inward  so  as  to  bring  the  back  of  the  forearms  outward, 
while  the  fingers  were  flexed  at  the  joints,  but  at  times  were  extended  and 
moved  slowly  in  a  way  characteristic  of  athetosis.  The  lower  extremities 
were  extended  at  all  the  joints,  the  feet  being  extended  in  talipes  equino- 
varus,  and  the  toes  were  flexed.  The  muscles  of  the  trunk  were  also  in- 
volved in  the  spasm.  At  first  the  left  side  was  the  more  severely  affected, 
but  afterward  the  spasm  became  equal  on  the  two  sides. 

The  electrical  irritability  of  the  muscles  was  normal,  and  there  was  no 
mechanical  excitability  of  the  nerves.  There  was  considerable  pyrexia  during 
the  more  severe  stage  of  the  disease.  The  boy  became  thoroughly  emaciated, 
and  died  from  exhaustion.  There  is  very  little  of  true  tetany,  and  still  less 
of  chorea  in  this  case  as  described  by  Gowers.  While  I  have  seen  no  case 
exactly  like  this  one  of  Gowers,  it  is  not  rare  to  find  irregular  spasmodic 
movements  of  all  sorts  that  may  remind  one  at  times  of  chorea,  at  times  of 
athetosis,  and  then  again  of  a  cataleptic  condition  during  febrile  disorders 
in  children. 

Escherich,  of  Vienna,  who  has  been  the  most  recent  and  a  most  careful 
student  of  tetany,  calls  attention  to  the  great  frequency  of  tetany  and  of 
tetanoid  disorders  in  young  children.     Of  eighty  cases  of  tetany  which  he 


TETANY.  165 

observed  in  the  Children's  Hospital,  the  majority  occurred  between  th-j 
of  three  to  thirty  months  and  he  is  inclined  to  infer  that  the  artificial  nourish- 
ment given  to  these  children  is  largely  responsible  for  the  development  of 
the  tetany.  Fully  one  per  cent,  of  all  the  children  under  three  years  of  age 
which  have  been  treated  in  Graz  were  suffering  from  tetany.  It  is  safe  to 
say  that  such  frequency  is  peculiar  to  certain  cities.  It  is  surely  very  differ- 
ent from  the  experience  of  American  pediatrists  and  neurologists.  I  wish 
specially,  however,  in  this  connection  to  allude  to  Escherich's  conclusions 
that  even  more  frequent  than  tetany  are  various  tetanoid  disorders  in  the 
earliest  years  of  life.  This  author  goes  to  the  extent  of  putting  laryngo- 
spasm,  muscular  spasms  and  eclampsia  into  a  series  characterized  by  those 
signs  which  would  lead  one  to  infer  that  all  of  them  were  tetanoid  in  charac- 
ter. The  present  writer  has  been  on  the  look-out  for  this  special  combina- 
ation  of  conditions  and  he  is  confident  that  in  America  this  combination  is 
not  a  very  common  one.  In  only  a  single  instance  has  he  seen  a  child  that 
presented  the  symptoms  of  laryngospasm  that  was  in  a  condition  of  muscular 
contracture  resembling  that  of  tetany  and  that  at  times  had  distinct  convul- 
sive seizures.  In  such  cases  the  tetanoid  character  of  the  condition  is  proved 
by  the  occurrence  of  Trousseau's  and  Chvostek's  symptoms  either  at  the  time 
of  the  spasm  or  during  the  period  of  latency.  Escherich's  observations 
should  encourage  us  to  look  for  the  existence  of  a  latent  tetany.  I  am 
confident,  however,  that  these  conditions  are  far  less  common  in  America 
than  they  are  in  Austrian  and  French  cities. 


CHAPTER   IX. 

HEADACHES. 

It  requires  no  little  skill  on  the  part  of  the  physician  to 
discover  the  true  cause  of  headaches.  They  do  not  consti- 
tute a  form  of  disease,  but  are  symptomatic  of  many  or- 
game  and  functional  conditions.  For  this  reason  we  must 
give  them  the  very  closest  study.  The  diagnosis  of  the 
kind  of  headache  in  the  adult  is  difficult  enough,  but  it  is 
doubly  so  in  the  case  of  children.  As  the  headaches  that 
come  on  in  early  life  are  frequently  continued  in  later 
years  and  give  rise  to  much  suffering,  it  is  particularly  im- 
portant that  an  effort  should  be  made  to  nip  them  in  the 
bud.  The  most  distressing  form  of  headache  and  the  one 
most  stubborn  in  its  resistance  to  treatment,  migraine,  will 
be  considered  in  a  separate  section.  The  remaining  forms 
may  be  classified  as  follows  : 

Headaches  due  to : 

i.  Anasmia  and  malnutrition. 

2.  Neurasthenia. 

3.  Transitory  hyperaemia,  as  it  occurs  in  heart  disease 
or  at  the  period  of  menstruation. 

4.  Gastric  disturbances. 

5.  Genital  irritation. 

6.  Ear  disease. 

7.  Organic  disease  of  the  brain  or  its  coverings  (includ- 
ing specific  disease). 

8.  The  prodromal  stage  of  acute  infectious  diseases. 

9.  Malaria. 

10.  Urasmic  poisoning. 

11.  Other  toxic  conditions. 

It  was  the  fashion  formerly  to  make  a  differential  diag- 
nosis of  the  forms  of  headaches   according   either  to  the 


HEADACHES. 


167 


character  of  the  headache  or  to  the  seat  of  the  distribution  of 
the  headache.  Thus  frontal  headaches  were  considered  to 
be  due  to  gastric  disturbances.  Occipital  headaches  were 
considered  to  be  almost  pathognomonic  of  uraemic  poison- 
ing ;  but  he  who  has  an  opportunity  of  seeing  a  large  number 
of  cases  of  headaches  in  the  adult,  as  well  as  in  children,  will 
soon  convince  himself  that  these  signs  are  often  misleading. 
I  have  seen  frontal  headaches  with  disease  of  the  kidneys, 
and  occipital  headaches  from  gastric  disturbances  and.  in 
cases  of  specific  disease,  of  tumor  of  the  brain,  etc.  The  diag- 
nosis of  the  kind  of  headache  can,  as  a  rule,  be  made  after 
carefully  examining  into  the  general  health  of  the  patient, 


Constipation        ) 
Caries  of  Incisor  J   \ 


Errors  of  Refraction 


Gastric  Dyspepsia  1 
Anaemia  j 


I  Neurasthenia 


-r-     Eye 

—     Decayed  Teeth 


I  Pharyngitis 
( Otitis  Media 


Fig.  51. — Location  of  Head-pains.     After  Dana  (slightly  altered).    Area  I.  Trigem- 
inus and  Facial  Nerve  Strands.     Area  II.  Upper  Four  Cervical  Nerve  Strands. 

and  endeavoring  to  find  what  the  fundamental  disturbance 
may  be.  It  will  be  better  for  us,  therefore,  to  take  the  eti- 
ological conditions  mentioned  above,  and  to  endeavor  to 
connect  with  each  condition  a  few  signs  that  are  generally 
associated  with  a  particular  form  of  headache. 

1.  Headaches  due  to  anaemia.  These  headaches  occur 
frequently  in  children  between  the  ages  of  eight  and  fifteen 
years ;  they  are  sometimes  frontal,  generally  vertical,  and 
are  described,  as  a  rule,  as  a  dull,  boring  headache,  and  in  the 
majority  of  cases  are  most  pronounced  early  in  the  morn- 
ing. They  are  frequently  attended  by  a  slight  vertigo  and 
a  feeling  of  faintness,  but  none  of  these  symptoms  is  char- 
acteristic enough  to  make  the  diagnosis  of  anaemic  head- 
ache unless  the  headaches  due  to  eye-strain,  to  neurasthenia, 


1 68  THE   NERVOUS  DISEASES   OF   CHILDREN. 

or  to  uraemic  conditions  can  be  excluded.  The  pallor  of 
the  conjunctivae,  which  may  be  obscured  by  a  catarrhal 
condition  very  frequent  in  children,  and  above  all  the  pal- 
lor of  the  gums  and  of  the  roof  of  the  mouth,  and  a  defi- 
ciency of  the  haemoglobin  are  the  signs  which  help  to  make 
the  diagnosis  ot  anaemic  headaches  certain  beyond  perad- 
venture 

The  result  of  treatment  will  also  furnish  corroborative 
evidence.  The  child  should  be  placed  at  rest,  fatigue 
should  be  avoided  in  every  possible  way,  and  its  diet  should 
be  nutritious  to  the  extreme.  It  is  important  not  only  to 
give  nutritious  food,  but  to  exclude  everything  that  is  of 
no  decided  value  to  the  child's  condition.  If  we  allow  an 
excess  of  fruit  or  of  sweetmeats  the  child  will  very  natu- 
rally look  for  these  rather  than  for  meat,  fish,  eggs,  oysters, 
which  should  constitute  its  main  diet,  with  a  slight  admixt- 
ure of  farinaceous  substances.  One  to  two  quarts  of  milk 
per  day,  three  or  four  eggs,  a  pound  or  more  of  meat  will 
be  all  of  far  more  benefit  to  the  child  than  any  number  of 
drugs.  They  may  help  a  little,  but  I  am  certain  that  it  is 
only  a  very  little;  and  if  there  is  any  question  of  the  child's 
power  of  assimilating  these  drugs  I  much  prefer  to  give 
them  up  rather  than  to  diminish  the  amount  of  food  that 
the  child  takes.  In  the  treatment  of  children's  diseases  the 
practical  necessity  of  prescribing  something  often  puts 
physicians  at  a  great  disadvantage.  The  era  of  rational 
therapeutics  has  not  yet  fully  dawned. 

These  anaemic  headaches  will  be  relieved  in  some  in- 
stances by  the  proper  use  of  hydrotherapy.  It  is  a  good 
plan  to  bathe  these  children  every  morning  immediately 
upon  rising.  Sponge  the  head  and  spine  first  with  warm 
water  and  then  with  cold,  allowing  the  water  to  drip  from  a 
little  height,  so  as  to  get  the  effect  of  the  impact  of  cool 
water.  After  this  they  should  be  briskly  dried  and  allowed 
at  once  to  dress.  In  the  case  of  very  weak  children  it 
would  be  better  to  have  the  child  take  a  little  nourishment, 
say  a  glass  of  hot  milk,  before  putting  it  through  this  pro- 
cedure. In  addition  to  this  the  child  should  be  given 
regular  calisthenic  exercises,  which  help  to  improve  the 
general  condition  and  to  develop  the  muscles,  and  to  pre- 


HEADACHES.  \CJ(J 

vent  the  accumulation  of  fat,  which  is  not  infrequent  in 
children  of  anasmic  disposition. 

The  meals  should  be  given  at  regular  intervals,  and  in 
cases  in  which  the  appetite  is  poor  the  plan  of  giving  small 
quantities  at  frequent  intervals,  say  every  two  hours,  is  to 
be  preferred  to  that  of  only  three  meals  during  the  day. 

The  most  useful  drug  to  be  employed  in  the  treatment  of 
these  headaches  is  caffeine  in  doses  of  one  to  two  grains 
every  hour,  or  at  least  three  times  a  day.  Black  coffee  will 
often  answer  the  same  purpose.  From  the  combination  of 
phenacetine  and  caffeine  I  have  seen  very  little  benefit,  and 
it  surely  has  no  advantage  over  the  administration  of  caf- 
feine alone,  and  is  possessed  of  the  disadvantage  that  phe- 
nacetine acts  unfavorably  upon  the  hearts  of  some  children. 
If  iron  is  to  be  given  let  it  be  administered  in  the  most 
easily  digested  form,  either  in  the  form  of  reduced  iron,  or 
in  one  of  the  many  preparations  of  the  peptonates  or  albu- 
minates of  iron  that  are  now  in  the  market.  Arsenic  can 
be  given  in  small  doses  of  from  three  to  four  drops  of  the 
Fowler's  solution  several  times  a  day.  In  cases  in  which  a 
general  tonic  effect  is  desired,  we  can  give  a  palatable  com- 
bination of  iron  and  quinine  with  small  doses  of  strychnine, 
from  one-eightieth  to  one-sixtieth  of  a  grain,  three  times  a 
day.  Why  phosphorus  should  be  recommended  by  some  I 
cannot  understand.  I  am  certain  that  no  one  can  conscien- 
tiously assert  that  he  has  seen  any  good  effect  from  its  use, 
and  whether  given  in  pill  form  or  in  the  form  of  Thomson's 
solution,  the  effect  in  my  experience  upon  cases  in  which  I 
have  known  it  to  be  tried  has  been  absolutely  nil.  The 
fewer  the  drugs  employed,  and  the  more  attention  is  paid  to 
the  hygiene  and  the  diet  of  the  child,  the  more  quickly 
this  anasmic  condition  will  disappear. 

2.  Headaches  due  to  neurasthenia.  These  headaches 
occur  so  frequently  in  persons  who  are  at  the  same  time 
anasmic,  that  the  treatment  of  one  cannot  be  considered 
apart  from  the  treatment  of  the  other.  In  anasmic  persons 
headaches  very  often  do  not  occur  until  they  have  been 
subjected  to  some  emotional  excitement,  or  have  passed 
through  some  fatiguing  ordeal.  These  headaches  can  be 
recognized  as   due   to   some  other  cause  than  to  anasmia 


I/O  THE  NERVOUS  DISEASES   OF   CHILDREN. 

alone  by  the  presence  of  other  symptoms  expressive  of  the 
neurasthenic  state,  such  as  slight  tremor  of  the  tongue  and 
fingers  and  the  exaggeration  of  the  deep  reflexes,  partic- 
ularly the  knee-jerks  ;  but  above  all  by  the  very  persistent 
location  of  these  headaches  on  the  crown  of  the  head,  and 
by  the  description  of  them  as  a  pressure  or  a  feeling  of  heat 
on  the  top  of  the  head.  This  description  of  the  headache 
is  as  characteristic  for  children  as  it  is  for  older  patients ; 
and  in  children  emotional  conditions,  the  strain  of  school 
work,  the  rivalry  between  classmates,  is  quite  as  apt  to  bring 
about  a  neurasthenic  state  and  to  cause  neurasthenic  head- 
aches as  are  the  more  serious  struggles  for  existence  in 
later  life. 

The  treatment  of  neurasthenic  headaches  may  be  con- 
ducted on  exactly  the  same  lines  as  was  referred  to  for 
those  due  to  anaemia.  The  hydrotherapeutic  procedures 
and  rest  are  by  far  the  most  important,  and  strychnine  and 
quinine  in  small  doses  will  be  of  far  more  benefit  than  if 
anaemia  were  the  only  cause  ;  but  a  cure  cannot  be  easily  ef- 
fected unless  all  exciting  conditions  are  removed,  and  to  that 
end  the  child  should  be  taken  from  school,  and  in  some 
instances  a  change  of  climate  and  of  home  surroundings 
may  be  necessary.  If  this  is  not  sufficient  a  short  rest  in 
bed  with  forced  feeding  may  be  attempted,  for  the  "  rest- 
cure  "  plan  is  as  efficient  in  children  as  it  is  in  adults. 

3.  Headaches  due  to  transitory  hyperasmia.  Fluctua- 
tions in  the  blood-supply  of  the  brain  sometimes  cause 
severe  headaches  in  children.  I  have  seen  many  young 
persons  who  after  some  unusual  excitement  or  intense  emo- 
tion develop  severe  headaches,  associated  either  with  deep 
flushing,  or  with  marked  pallor  of  the  face  and  with  cold 
extremities.  In  others,  again,  persistent  headaches  come  on 
after  an  acute  exhausting  disease,  in  whom  it  must  be  sup- 
posed that  if  there  is  any  transitory  hyperaemic  condition 
it  is  rapidly  followed  by  a  lack  of  blood  in  the  vessels  of 
the  brain  and  its  coverings.  A  chronic  hyperaemic  condi- 
tion is  probable  in  children  afflicted  with  heart  disease,  who 
are  often  subject  to  periods  of  painful  headaches,  and  a 
similar  explanation  must  be  sought  for  in  young  girls  just 
beginning  to  menstruate,  who  have  severe  headaches  for 


HEADACHES. 


171 


several  days  preceding- and  during  each  menstrual  period. 
While  such  headaches  are  often  strictly  periodic  they  are 
very  different  from  ordinary  migraine,  and  can  be  distin- 
guished from  the  latter,  too,  by  the  late  onset  of  the  trouble 
and  by  their  disappearance  as  soon  as  the  menstrual  func- 
tion has  been  properly  established. 

4.  Headaches  due  to  gastric  disturbance  are  a  very 
common  occurrence  in  children  of  all  ages.  They  are 
generally  frontal,  sometimes  frontal  and  vertical.  They 
come  on  suddenly  and  persist,  as  a  rule,  until  the  gastric 
condition  has  been  relieved.  These  headaches  are  easily 
recognized  by  the  symptoms  associated  with  them,  viz., 
fetid  breath,  coating  of  the  tongue,  distress  in  the  epigas- 
tric region,  flatulency,  and  constipation  or  diarrhoea.  The 
diagnosis  is  easily  corroborated  by  the  effects  of  treatment, 
a  single  dose  of  oil  or  of  calomel  being  sufficient,  as  a  rule, 
to  dispel  them  until  the  next  serious  error  in  diet  is  com- 
mitted. 

5.  Genital  irritation  is  referred  to  by  some  writers  as  a 
cause  of  severe  headaches.  Seguin  refers  to  a  case  of  oc- 
cipital headache,  the  worst  he  ever  saw,  which  was  cured 
by  circumcision.  I  have  not  seen  such  a  case,  but  I  do 
not  wish  to  doubt  their  occurrence,  and  if  the  physician  is 
convinced  of  the  cause  of  the  disturbance  the  remedy  is 
close  at  hand.  In  older  boys,  and  even  in  girls,  headaches 
are  not  infrequently  due  to  self-abuse.  The  general  nervous 
condition  of  the  patient  under  these  circumstances  calls 
for  treatment  even  more  distinctly  than  do  the  headaches 
which  are  associated  with  a  general  restlessness,  with  pains 
in  the  back,  with  irritability,  and  in  severe  cases  with  a 
tendency  to  mental  apathy  or  even  dementia.  There  is 
every  reason  to  look  for  this  etiological  factor  in  any  case 
of  persistent  headaches  in  children  between  the  ages  of  six 
and  fifteen  years.  The  treatment  of  the  headaches  under 
these  circumstances  is  not  an  easy  matter,  and  practically 
they  can  be  cured  only  by  the  closest  watchfulness  on  the 
part  of  parent  or  nurse. 

6.  The  headaches  associated  with  ear  disease  are  char- 
acterized by  intense  pain,  located  either  in  the  mastoid  or 
in  the  temporal  region,  and  are  most  frequently  present  in 


172  THE  NERVOUS  DISEASES   OF   CHILDREN. 

the  earlier  stages  of  the  disease,  before  the  formation  of 
pus.  One  of  the  worst  cases  of  this  sort  that  I  have  ever 
seen  was  in  a  little  girl,  two  years  of  age,  who  was  un- 
manageable and  showed  by  her  movements  that  she  was  in 
great  distress.  Every  touch  on  the  head  was  followed  by 
a  shrill  cry,  and  the  region  of  the  ear  was  so  sensitive  that 
the  slightest  touch  with  the  finger  seemed  to  cause  intense 
agony.  No  treatment  was  of  any  avail  until  after  a  few 
days  the  pus  was  freely  discharged  through  the  outer  canal, 
and  from  that  moment  every  trace  of  headache  seemed  to 
have  disappeared.  The  child's  behavior  is  exactly  like  that 
of  an  adult  with  acute  ear  disease  ;  it  is  true,  however,  that 
this  special  cause  of  headache  is  often  discovered  only  by 
the  merest  accident.  The  practitioner  and  the  specialist 
in  ear  diseases  will  meet  with  these  cases  so  often,  particu- 
larly in  connection  with  the  acute  infectious  diseases,  that 
they  should  be  on  their  guard.  The  cure  depends  entirely 
upon  prompt  treatment  of  the  ear  condition. 

7.  Headaches  due  to  organic  disease  of  the  brain  or  its 
coverings  should  be  suspected  in  every  case  in  which  the 
pain  is  persistent  and  strictly  localized.  The  pain  may 
vary  a  little  according  to  the  position  of  the  head,  but  it  is 
present  whether  the  child  be  sitting  up  or  lying  down,  and 
is  always  elicited  by  the  gentlest  percussion  of  the  skull. 
The  headaches  constitute  a  most  valuable  symptom  in  the 
diagnosis  of  tumors  of  the  brain  or  of  the  meninges,  and 
in  cases  of  meningeal  inflammation  from  any  and  every  cause. 
Thus  after  minor  or  greater  injuries  to  the  head  the  onset 
of  intense  pain,  particularly  at  the  seat  of  external  injury, 
must  lead  one  to  suspect  the  development  of  a  morbid  con- 
dition at  this  point.  If  due  to  tumor,  the  diagnosis  can  be 
strengthened  by  the  discovery  of  other  symptoms,  which 
are  bound  to  arise  sooner  or  later.  These  are  vertigo, 
nausea,  and  disturbances  of  vision  due  to  optic  neu- 
ritis. If  a  traumatic  meningitis  is  the  cause  of  pain  in 
the  head,  slight  rigidity  of  the  neck,  and  possibly  an  in- 
equality and  immobility  of  the  pupils,  will  help  to  prove 
the  diagnosis. 

Among  the  organic  headaches  we  might  also  include 
those  due  to  specific  disease;  but  these  headaches,  if  I  may 


HEADACHES.  1 73 

trust  my  own  experience,  are  relatively  rare  in  cases  of 
hereditary  syphilis  or  in  syphilis  acquired  at  a  very  early 
date.  They  are  surely  not  nearly  as  constant  nor  so  im- 
portant a  symptom  as  are  the  specific  headaches  of  later 
years.  The  diagnosis  should  be  made  with  great  reserve, 
and  only  if  other  symptoms  are  present  which  point  to  an 
active  syphilitic  process. 

8.  The  headaches  which  mark  the  prodromal  stage  of 
acute  infectious  diseases  often  give  rise  to  very  serious  er- 
rors in  diagnosis,  and  are  occasionally  suspected  to  be  symp- 
toms of  cerebral  tumor,  or  possibly  of  meningitis.  In  the 
prodromal  stages  of  typhoid  and  scarlatina,  and  of  diph- 
theria, these  headaches  are  very  common  indeed  ;  but  the 
possibility  of  such  a  cause  for  headaches  should  be  enter- 
tained if  they  are  associated  with  a  general  malaise  and 
with  slight  rise  in  temperature.  There  is  no  need  of  dis- 
cussing the  treatment  of  this  special  form.  Unless  one 
chooses  to  apply  cold  cloths,  or  an  icebag,  or  possiblv  to 
give  small  doses  of  phenacetine,  there  is  nothing  else  to  do 
but  to  wait  for  further  developments,  and  to  treat  the  more 
serious  disease  of  which  the  headaches  constitute  the  pro- 
dromal stage. 

9.  Malarial  headaches  are  not  observed  nearly  so  often 
in  this  climate  as  in  the  more  southern  States,  and  in 
those  countries  in  which  severe  types  of  malaria  are  prev- 
alent. Malarial  headaches  are  almost  invariably  neuritic  in 
character.  The  supraorbital  and  infraorbital  points  are 
painful,  and  the  entire  head  may  be  sensitive  to  touch. 
There  is  a  distinct  periodicity  in  the  development  of  these 
headaches,  or  if  the  headaches  are  continuous  there  are  at 
least  periodic  exacerbations. 

In  making  a  diagnosis  of  malarial  headaches  I  follow  the 
practice  of  examining  the  spleen  and  the  blood,  and  not 
contenting  myself  with  the  diagnosis  unless  I  can  prove 
the  existence  of  enlarged  spleen,  or  the  presence  of  the  Plas- 
modium in  the  blood.  If  such  evidence  is  obtained,  a  few 
large  doses  of  quinine — from  five  to  ten  grains  several  times 
a  day,  according  to  the  age  of  the  child — will  prove  the  best 
cure. 

10.  Ursemic  headaches  are  not  as  frequent  in  children  as 


174  THE  NERVOUS  DISEASES    OF   CHILDREN. 

in  the  adult.  These  headaches  are  generally  occipital,  and 
are  associated  with  slight  disturbances  of  vision,  with  ver- 
tigo, nausea,  and  sometimes  with  epigastric  pain.  The  con- 
dition of  the  kidneys  should  be  carefully  determined,  if 
severe  headaches  arise  during  or  after  any  of  the  acute  in- 
fectious diseases  ;  above  all,  in  scarlatina  and  diphtheria, 
which  are  known  to  be  followed  by  renal  complications. 

ii.  Other  toxic  headaches  deserve  special  mention. 
They  are  not  frequent,  but  do  occur  often  enough  to  make 
it  necessary  to  examine  for  this  possible  cause.  Among 
toxic  substances  lead  is  most  easily  productive  of  severe 
headaches.  The  poison  is  apt  to  be  taken  into  the  system 
not  only  with  milk  that  is  kept  in  leaden  jars,  but  with 
drinking-water  passing  through  leaden  pipes,  and  I  have 
known  it  to  result  from  chewing-gum,  and  all  sort  of  vile 
sweetmeats  that  are  wrapped  up  in  attractive  papers,  the 
children  licking  these  papers  in  order  that  they  may  get 
the  full  value  of  their  purchase.  Some  years  ago  I  saw  at 
my  clinic  a  young  girl,  of  about  fifteen  years,  suffering  from 
headaches,  and  from  a  typical  lead  palsy,  who  had  evidently 
taken  the  poison  into  her  system  in  the  course  of  her  daily 
work,  which  consisted  of  gluing  together  the  paper  in 
which  chewing-gum  was  wrapped.  She  was  in  the  habit 
of  putting  her  tongue  to  the  glue  and  the  paper  instead  of 
moistening  the  former  with  a  sponge. 

I  have  reserved  for  the  last,  the  consideration  of  head- 
aches due  to  eye-strain.  Not  that  I  think  them  the  least 
frequent,  but  because  in  my  opinion  undue  importance  has 
been  attached  to  them.  The  cases  that  are  due  to  eye-strain 
are  those  in  which  the  headaches  come  on  after  reading,  or 
in  studying.  They  may  persist  for  some  time  after  the 
effort  is  made,  but  frequently  disappear  after  the  effort  is 
relaxed.  Serious  errors  of  refraction  may  be  the  cause  of 
headaches,  and  of  continuous  headaches,  even  though  no 
effort  be  made  to  use  the  eyes ;  but  I  have  seen  headaches 
persist  so  frequently  after  the  fitting  of  glasses  by  the  most 
competent  oculists  that  I  am  firmly  convinced  that  eye- 
strain is  the  sole  cause  of  headaches  in  relatively  few  in- 
stances. 

These  headaches  are  located  in  the  frontal  region,  be- 


MIGRAINE.  1/5 

tween  and  over  the  eyebrows  (Fig.  51);  in  some  instances 
with  evident  eye-strain  the  headache  is  referred  to  the  occi- 
put. I  am  thoroughly  in  favor  of  giving  every  child  the 
benefit  of  the  doubt,  and  of  making  a  careful  examination 
into  the  condition  of  the  eyes ;  but  I  wish  to  protest  against 
the  excessive  enthusiasm  of  the  day  which  implies  that  if 
the  slightest  error  of  refraction  is  discovered  in  a  child  the 
error  must  be  the  cause  of  all  ills.  What  is  claimed  for 
headaches  is  claimed  under  similar  conditions  for  epilepsy 
and  for  chorea. 

The  above  considerations  will  convince  the  student  that 
the  diagnosis  of  headaches  is  no  easy  matter.  To  make  an 
accurate,  or  even  plausible,  diagnosis  of  this  condition  is 
one  of  the  most  difficult  tasks  in  neurology.  It  can  be  done 
properly  only  by  a  careful  consideration  of  the  general  con- 
dition of  the  patient,  of  the  symptoms  associated  with  the 
headaches,  and  of  the  patient's  health  before  and  after 
the  headache  has  been  developed. 

MIGRAINE. 

Among  the  neuroses  of  early  youth  few  are  more 
troublesome  or  more  interesting  than  migraine,  or  "  sick 
headache."  While  the  disease  does  not,  as  a  rule,  attain  its 
full  development  until  the  age  of  puberty  or  later,  it  begins 
so  often  in  the  earlier  years  of  life  that  it  belongs  very 
properly  to  the  special  subjects  of  this  book.  The  symp- 
toms of  the  disease  as  it  occurs  in  children  are  so  very  much 
like  the  adult  form  that  in  describing  one  we  picture  the 
other. 

Hemicrania  (megrim),  or  sick  headache,  is  characterized 
by  occasional  attacks  of  intense  headaches,  frequently  unilat- 
eral, which  are  associated  with  a  feeling  of  nausea,  or  with 
vomiting.  The  unilateral  headache  is  by  far  the  more  im- 
portant symptom  of  the  two,  for  in  many  cases  the  charac- 
teristic headache  is  present  for  months  and  years  without 
nausea  ever  being  associated  with  it,  though  I  must  record 
cases  from  my  own  experience  in  which  periodic  attacks  of 
nausea  have  occurred  in  children  without  any  headaches. 
Such  attacks  I  have  interpreted  to  be  the  equivalent  of  or- 


176  THE  NERVOUS  DISEASES   OF   CHILDREN. 

dinary  migraine.  The  correctness  of  this  view  has  been 
proved  by  the  occasional  occurrence  of  attacks  in  which 
both  nausea  and  headaches  were  present,  and  by  the  fact 
that  the  ordinary  treatment  for  migraine  and  no  other 
helped  to  dispel  these  peculiar  gastric  attacks.  Barring 
such  occasional  cases,  it  is  better  for  us  to  consider  the 
typical  attack  in  which  neither  headache  nor  nausea  is 
wanting. 

In  this  disease  we  find  a  number  of  very  important  symp- 
toms associated  with  headache.  Peculiar  visual  disturbances 
constitute  a  prominent  feature  of  the  disease  ;  these  have 
been  variously  described  by  many  sufferers,  and  amount, 
as  a  rule,  to  a  temporary  and  partial  loss  of  sight  during  the 
attack  ;  or  there  may  be  every  possible  form  of  visual  dis- 
turbance, from  simple  balls  of  fire  to  distinct  figures,  which 
appear  as  regularly  in  the  attacks  of  migraine  as  similar 
phenomena  do  in  attacks  of  epilepsy.  In  many  cases  a  sim- 
ple dark  spot  is  observed  ;  in  others,  flashes  of  lightning 
that  surprise  the  patient  are  the  first  symptoms  of  a  full- 
fledged  attack  of  migraine.  A  young  patient  of  mine  would 
regularly  see  a  bright  zigzag  line,  which  she  compared  to 
a  distant  staircase ;  as  soon  as  she  seemed  actually  to  ap- 
proach the  stairs  intense  headaches  would  set  in,  and  she 
would  feel  dizzy,-  but  there  was  never  loss  of  consciousness, 
and  nothing  resembling  an  epileptic  attack.  The  patient 
would  then  pass  through  a  typical  attack  of  migraine,  which 
would  last  for  several  hours,  and  during  this  entire  time 
would  exhibit  very  marked  photophobia.  In  some  cases 
other  special  senses  are  affected.  Tinnitus  is  much  more 
frequent,  on  the  whole,  than  any  other  form  of  sensory 
disturbance  excepting  those  of  vision  noted  above. 

Disturbances  of  sensation  in  the  limbs  are  a  frequent  ac- 
companiment of  migraine.  These  sensations  generally  take 
the  form  of  tingling,  of  pins  and  needles,  or  of  burning  sen- 
sations. In  some  instances  there  is  a  general  numbness, 
very  much  like  the  numbness  that  precedes  an  attack  of 
epilepsy  ;  the  sensations,  however,  last  very  much  longer 
than  the  sensory  aura  would,  and  can,  of  course,  be  distin- 
guished from  the  latter  by  the  entire  absence  of  any  typical 
clonic  movements.     Motor  symptoms  are  not  present  as  a 


MIGRAINE.  \-j 

rule.  As  there  is  a  close  association,  at  times,  between  mi- 
graine and  epilepsy  it  is  possible  that  we  may  see  cases 
every  now  and  then  in  which  the  auras  resemble  very  much 
those  that  precede  an  attack  of  migraine,  but  the  clonic 
movements  are  characteristic  of  epilepsy. 

Temporary  aphasia  I  have  met  with  as  an  accompani- 
ment of  an  attack  of  migraine.  I  recall  the  case  of  a  young 
girl,  ten  years  old,  who  had  inherited  migraine  from  her 
mother,  and  who  greatly  alarmed  her  parents  by  the  sudden 
development  of  aphasia  in  connection  with  an  attack  of  mi- 
graine. The  girl  when  spoken  to  was  able  to  mumble  a  few 
words  indistinctly,  but  could  not  find  the  word  she  wished 
to  say.  She  was  in  intense  pain  and  extremely  irritable, 
but,  after  a  good  night's  rest,  the  headache  had  disappeared 
and  with  it  the  aphasia.  This  aphasia  is  associated  with 
right-sided  hemiplegia  in  right-handed  persons.  The  occur- 
rences during  these  attacks  of  migraine  adhere  closely  to 
the  physiological  laws  of  the  cortex,  and  we  may  anticipate 
what  we  have  to  say  upon  the  pathology  of  the  subject  to 
the  extent  of  implying  that  the  sequence  of  symptoms  evi- 
dently proves  that  the  entire  motor  district  of  the  brain 
must  be  affected  by  the  temporary  defect  in  its  blood- 
supply. 

Vertigo  is  another  symptom  that  is  associated  with  the 
headaches,  at  times  preceding  it,  at  other  times  following 
it.  The  vertigo  is  not,  as  a  rule,  as  marked  as  it  is  in  Me- 
niere's disease  or  in  some  organic  diseases  of  the  brain,  but 
it  is  quite  sufficient  to  make  the  patient  unsteady  on  his 
feet,  and  to  give  rise  to  a  great  deal  of  discomfort. 

The  aspect  of  the  patient  varies  considerably  during  the 
attack.  In  some  there  is  distinct  pallor  of  the  face  and  a 
feeling  of  coldness  in  the  extremities.  The  eyeball  may 
seem  a  trifle  retracted,  the  vessels  of  the  conjunctiva  may 
be  engorged,  and  the  pupils  may  be  contracted.  In  other 
cases  the  face  may  be  extremely  flushed,  the  pupils  dilated 
rather  than  contracted,  and  the  ear  distinctly  reddened. 
These  two  distinct  conditions  are  often  present  in  one  and 
the  same  attack,  and,  in  rare  instances,  the  one-half  of  the 
face  may  present  pallor  with  its  associated  symptoms, 
whereas  the  other  half  presents  a  flushed  condition  with 


178  THE  NERVOUS  DISEASES   OF  CHILDREN. 

the  symptoms  that  go  with  it.  The  latter  symptoms  are 
evidently  of  the  paretic  order  and  the  former  of  the  spas- 
tic, and  both  are  evidences  of  a  change  in  the  sympathetic 
nerves.  Sweating  of  one  side  of  the  face  has  also  been  ob- 
served, as  well  as  retardation  of  the  pulse  during  the  parox- 
ysms. On  examination  during  an  attack,  I  have  found  the 
various  points  of  the  trigeminal  nerve  quite  as  sensitive  as 
in  the  milder  cases  of  trigeminal  neuralgia.  In  some  pa- 
tients— and  this  is  particularly  true  of  children — the  entire 
face  and  head  is  so  sensitive  that  the  patient  can  scarcely 
bear  to  rest  the  head  on  a  pillow,  and  I  have  known  one 
patient  who  insisted  on  "  walking  off  "  the  headache  rather 
than  to  rest  the  head  against  anything  during  the  parox- 
ysm. 

Gowers  refers  to  a  transitory  pyrexia  during  an  attack 
of  migraine  in  children.  I  have  often  taken  the  tempera- 
ture during  attacks,  impelled  to  do  so  by  a  flushed  condition 
of  the  face,  but  have  never  found  any  elevation  above  ioo° 
F.  If  a  higher  temperature  is  present  I  should  be  disposed 
to  look  for  some  other  condition,  say  some  gastric  disturb- 
ance, as  a  possible  cause  both  of  the  migraine  and  of  the 
fever. 

Etiology. — The  hereditary  predisposition  to  migraine 
is  too  manifest  to  be  denied  for  a  single  moment.  Not  only 
do  children  of  mothers  who  are  thus  affected  inherit  the 
disease,  but  it  also  occurs  in  the  progeny  of  persons  afflicted 
with  other  grave  forms  of  functional  nervous  disturbance. 
Among  these  epilepsy,  hysteria,  and  hystero-epilepsy  are 
the  most  potent  sources.  The  disease  usually  sets  in  in  the 
earlier  years  of  life.  Some  begin  between  the  fifth  and  tenth 
years,  a  fair  number  of  the  cases  between  ten  and  twenty 
years,  but  the  majority  set  in  between  twenty  and  thirty 
years. 

The  female  sex  is  evidently  more  disposed  to  the  disease 
than  males. 

The  first  manifestations  of  migraine  are  generally  excited 
by  emotional  disturbances,  by  overwork,  or  worriment.  In 
not  a  few  instances  I  have  known  the  rivalry  among  class- 
mates to  have  been  the  final  cause  of  the  development  of 
migraine ;  in  others,  some  severe  gastric  disturbance  has 


MIGRAINE.  1 79 

been  the  starting-point  of  the  entire  trouble,  but  it  should 
be  remembered  that,  if  a  predisposition  did  not  exist,  the 
derangement  of  the  stomach  might  have  produced  a  tem- 
porary headache,  but  would  not  have  started  a  series  of 
attacks  of  migraine.  After  the  first  attack  an  interval  of 
some  weeks,  or  months,  may  intervene  before  a  second 
seizure  takes  place ;  but  some  form  of  periodicity  is  soon 
established  in  the  majority  of  the  cases,  and  in  many,  even 
in  young  children,  the  attacks  are  apt  to  return  at  stated 
intervals — every  two,  every  four  weeks,  or  every  second  or 
third  month.  It  is  with  migraine  very  much  as  it  is  with 
epilepsy,  that  the  slightest  disturbance  of  the  physical  or- 
ganism is  sufficient  to  develop  an  attack.  It  is,  therefore,  of 
the  greatest  importance  in  migraine,  as  in  epilepsy,  that  the 
most  careful  attention  should  be  paid  to  the  general  hygi- 
enic and  dietetic  management  of  the  child. 

Pathology. — We  cannot  expect  to  demonstrate  actual 
changes  in  the  brain,  or  in  any  other  part  of  the  central  ner- 
vous system  in  a  person  afflicted  with  migraine,  for  persons 
so  afflicted  rarely  die  after  a  paroxysm,  and  the  attack  of  mi- 
graine evidently  represents  a  transitory  change  ;  but  since 
the  anatomical  basis  is  wanting,  the  opportunity  for  theo- 
retical speculations  regarding  the  pathology  of  the  disease 
is  all  the  greater,  and  has  been  improved  by  numerous 
writers.  There  can  hardly  be  a  doubt  that  changes  in  the 
blood-supply  of  the  brain  or  its  coverings  are  primarily 
responsible  for  the  symptoms  of  migraine.  There  is  also 
reason  to  believe  that  the  sympathetic  nervous  system  is 
largely  involved  in  this  disease.  According  to  the  varying 
conditions  present  many  neurologists  are  inclined  to  sup- 
pose that  in  some  instances  we  have  an  angio-spastic  condi- 
tion, in  others  an  angio-paralytic  state,  and  if  we  wish  to 
imply  our  belief  in  the  role  played  by  the  sympathetic 
nerves  we  can  speak  of  "  sympathetico-tonic  "  or  "  sympa- 
thetico-paralytic  "  forms.  The  behavior  of  the  superficial 
blood-vessels  in  the  two  forms  of  migraine  lends  reasonable 
coloring  to  this  view  of  the  vasomotor  origin  of  migraine. 

Some,  not  satisfied  with  this  vascular  theory,  argue  that 
there  must  be  some  inherent  alteration  in  the  nerve-cells  of 
the  brain,  but  no  sufficient  reason  is  given  why,  if  such  de- 


l80  THE  NERVOUS  DISEASES   OF   CHILDREN. 

rangement  exists,  there  should  be  such  violent  periodic  ex- 
acerbations  of  all  the  symptoms.  To  say  that  the  symp- 
toms are  due  to  a  "  nerve  storm  "  is  merely  substituting  a 
vague  term  for  a  vague  conception ;  yet  we  must  acknowl- 
edge that  a  peculiarity  in  the  structure  or  in  the  function 
of  the  cortical  cells  may  be  present  in  these  cases,  and  that 
changes  in  the  vasomotor  apparatus  are  sufficient  to  pro- 
duce the  phenomena  of  migraine  in  a  person  whose  nervous 
system  is  thus  altered,  whereas  the  same  vasomotor  changes 
would  be  entirely  insufficient  to  produce  any  such  symptoms 
in  persons  whose  brain -cells  are  altogether  normal.  It 
seems  to  me,  therefore,  that  we  must  rely  upon  these  two 
causes  for  an  explanation  of  the  disease  ;  surely  one  cause 
alone  would  not  be  sufficient  to  explain  all  the  phenomena. 
We  are  constantly  losing  sight  of  the  co-operation  of  forces 
and  of  causes  that  produce  disease,  and  are  hampered  so 
frequently  by  the  supposition  that  we  must  make  out  a 
single  cause  or  none. 

The  disturbances  of  vision,  as  well  as  the  temporary 
aphasia  occurring  in  some  cases,  prove  conclusively  that 
the  cortical  centres  are  involved  in  the  disease.  The  visions 
of  migraine  can  be  regarded  as  a  symptom  of  irritations 
the  hemianopsia  as  a  symptom  pointing  to  temporary  paral- 
ysis of  the  functions  of  the  visual  centres.  Whether  a  de- 
rangement of  the  cells  or  some  other  change  in  the  consti- 
tuent parts  in  these  centres  is  responsible  for  the  loss  of 
function  cannot  yet  be  proved  or  denied. 

The  relation  of  migraine  to  epilepsy  makes  it  also  more 
probable  that  some  primary  peculiarity  of  the  cortical  cells 
is  responsible  for  this  painful  affection.  The  resemblance 
is  so  close,  and  the  sequence  of  the  two  diseases  so  strik- 
ing, that  the  two  forms  of  disease  may  possibly  represent 
a  different  degree  of  affection  of  the  cortical  structure. 

As  for  the  headaches  of"  migraine,  they  can  be  best  ex- 
plained, it  seems  to  me,  on  the  supposition  of  a  marked 
distention  of  the  blood-vessels  of  the  coverings  of  the  brain  ; 
and  the  fact  that  various  points  in  the  face  are  as  painful  as 
they  are  in  typical  trigeminal  neuralgia,  would  lead  to  the 
inference  that  the  trigeminal  nerve,  as  it  passes  from  the 
brain  outward,  is  affected  by  this  general  hyperasmic  con- 


MIGRAINE.  j  8l 

dition.  The  nausea  and  vomiting  are  an  expression  of  gen- 
eral cerebral  disturbances,  such  as  is  seen  in  cases  of  gross 
disease  anywhere  in  the  brain,  particularly  in  the  lower 
centres  in  the  pons  and  medulla,  and  such  as  we  sometimes 
find  in  cases  of  simple  cerebral  shock.  I  cannot  see  suffi- 
cient reason  to  connect  these  symptoms  directly  with  an 
affection  of  the  sympathetic  nerve. 

Within  the  last  few  years  much  has  been  made  of  ocular 
insufficiencies  as  a  possible  cause  of  migraine.  This  matter 
has  been  much  overdone,  thanks  to  the  labors  of  Dr.  Stevens 
and  others.  As  a  neurologist  I  could  pass  over  the  rather 
elaborate  discussion  that  has  been  held  on  this  subject, 
were  it  not  for  the  fact  that  so  high  an  authority  as  Dr. 
Seguin  has  given  his  approval  to  this  special  doctrine  in 
his  lectures  on  the  treatment  of  neuroses  {New  York  Med- 
ical Journal,  1890).  Dr.  Seguin  states  that  he  has  not  met 
with  a  case  of  migraine  in  a  person  with  normal  eyes,  al- 
though he  has  been  told  of  two  or  three  by  oculists  of  good 
repute.  He  implies,  furthermore,  that  if  the  matter  has 
been  overlooked  in  the  majority  of  cases  of  migraine,  it  is 
because  the  ocular  examination  has  not  been  a  thorough 
one.  He  argues,  furthermore,  that  the  ocular  origin  of 
migraine  is  made  probable  by  the  remarkable  fact  that  in 
many  persons  of  both  sexes  the  attacks  diminish  and  then 
cease  between  the  ages  of  forty  and  fifty  years,  at  the 
time  the  power  of  accommodation  becomes  exhausted,  and 
a  large  part  of  the  unconscious  strain  which  has  been  going 
on  from  early  youth  is  removed.  Seguin  implies  still  further 
that  migraine  is  hereditary,  chiefly  because  ocular  defects 
are  hereditary.  The  fallacy  of  these  arguments  seems  to 
me  to  be  evident  enough  from  the  experience  many  of  us 
have  had,  that  the  eyes  are  normal  according  to  the  exami- 
nation of  competent  oculists  in  many  children  who  have 
migraine,  and  that  the  attacks  continue  long  after  the  ocu- 
lar difficulty  has  been  corrected  by  glasses.  We  are  willing 
to  concede  that  these  ocular  difficulties  have  an  important 
bearing  upon  headaches  in  general,  and  may  influence  the 
occurrence  of  attacks  in  those  who  have  inherited  migraine; 
but  far  stronger  evidence  will  have  to  be  advanced  before 
it  will  be  safe  to  adopt  Seguin's  conclusions,  and  some  other 


1 82  THE   NERVOUS  DISEASES    OF   CHILDREN. 

sufficient  reason  will  have  to  be  given  for  the  action  of 
mydriatics,  such  as  belladonna,  atropine,  hyoscyamia,  and 
cannabis  indica,  than  the  effect  these  have  upon  the  accom- 
modative effort.  In  my  own  experience  with  the  drugs 
mentioned,  cannabis  indica  is  the  only  one  which  has  any 
decided  effect  upon  the  course  of  migraine  or  upon  single 
attacks. 

Diagnosis. — The  diagnosis  of  migraine  is  easily  made. 
The  early  onset  of  the  disease,  the  periodic  attacks,  and  the 
character  of  the  headaches  will,  as  a  rule,  leave  little  room 
for  doubt.  It  is  only  in  those  cases  in  which  a  distinct  sen- 
sory aura  exists  that  a  confusion  with  epilepsy  might  arise, 
but  the  preservation  of  consciousness  and  the  entire  absence 
of  clonic  movements,  and  the  duration  of  the  single  attack, 
will  help  to  distinguish  migraine  from  epilepsy.  It  may 
be  a  little  more  difficult  at  times  to  distinguish  between 
migraine  and  headaches  due  to  some  other  cause,  but  this 
difficulty  will  cease  as  soon  as  the  periodicity  in  the  occur- 
rence of  the  headaches  has  been  noted,  and  every  other 
form  of  headache  has  been  ruled  out  by  the  method  of  ex- 
clusion. I  have  found  some  little  difficulty  also,  particu- 
larly in  young  children,  to  distinguish  between  migraine 
and  ordinary  trigeminal  neuralgia,  for  owing  to  the  sensi- 
tiveness of  younger  patients  it  is  not  so  easy  to  determine 
whether  there  are  distinct  painful  points,  or  whether  the 
whole  face  is  painful,  merely  as  a  result  of  the  fear  of  ex- 
amination ;  but  the  long  and  free  interval  between  attacks, 
together  with  the  periodic  occurrence  of  the  headaches  in 
migraine,  will  serve  to  distinguish  this  form  from  typical 
trigeminal  neuralgia.* 

PROGNOSIS. — The  prognosis  of  migraine  is  entirely  fa- 
vorable as  regards  life,  but  not  so  promising  with  reference 
to  the  cure  of  the  disease.  It  is  a  matter  of  common  ex- 
perience that  a  person  who  has  inherited  migraine  will  be 
subject  to  the  disease  for  a  long  number  of  years,  until  it 
begins  to  disappear  with  age.  It  is  a  satisfaction,  however, 
to  be  able  to  assure  the  patient  that  under  proper  treatment 
the  attacks  may  be  either  inhibited  or  their  severity  may  be 
diminished.     The  only  grave  feature  about  the  disease  is 

*  Trigeminal  neuralgia  is  a  rare  affection  in  children. 


MIGRAINE.  I83 

the   possible  development   of   migraine  into  epilepsy,  but 
even  this  is  an  infrequent  occurrence. 

Treatment. — In  the  treatment  of  migraine  two  distinct 
objects  must  be  kept  in  view.  First — the  cure,  if  possible, 
of  the  disease  ;  secondly — the  amelioration  of  the  attacks. 

The  disease  often  defies  the  skill  of  the  most  experienced 
practitioner.  As  in  epilepsy,  no  effort  should  be  spared 
to  accustom  the  patient  to  regular  hygienic  and  dietetic 
habits.  It  is  important  that  the  child  thus  afflicted  should 
sleep  in  a  well-ventilated  room  ;  that  it  should  have  regular 
and  moderate  physical  exercise,  a  point  of  the  greatest  im- 
portance in  those  who  have  inherited  gouty  or  lithaemic 
tendencies.  The  child  should  be  relieved  also,  as  far  as 
possible,  of  all  mental  and  physical  strain.  While  the  dis- 
ease is  at  its  worst  the  patient  should  be  kept  from  school, 
and  all  close  application  to  study  should  be  avoided.  If  it 
can  be  shown  that  the  effort  of  reading  or  of  studying  helps 
to  bring  on  an  attack,  even  study  at  home  and  reading  may 
have  to  be  prohibited.  As  a  matter  of  fact,  however,  a 
little  mental  occupation  is  often  to  be  preferred  to  idleness, 
which  permits  the  child  to  wait  for  the  onset  of  an  attack 
and  often  induces  distinct  hypochondriacal  tendencies.  My 
own  experience  has  been  very  largely  in  favor  of  mod- 
erate employment  of  mind,  which  helps  to  prove  to  the 
child  that  its  lot  is  not  a  worse  one  than  that  of  other  chil- 
dren, and  helps  also  to  avoid  the  idea  of  invalidism  which  is 
so  frequent  in  children,  even  in  those  who  do  not  for  one 
reason  or  another  care  to  simulate  disease. 

If  the  child  is  anaemic,  or  if  it  exhibits  scrofulous  ten- 
dencies, iron  in  any  of  its  various  forms,  arsenic  and  quinine 
in  tonic  doses,  as  well  as  cod-liver  oil,  are  indicated.  Phos- 
phorus is  of  so  little  use  that  we  can  disregard  it  alto- 
gether. 

A  single  attack  can  be  relieved  best  by  putting  the 
child  in  bed  during  the  period  of  the  attack,  and  in  a  dark- 
ened room.  On  the  whole  mere  rest  in  bed  is  as  important 
a  factor  as  any  in  the  treatment  of  the  attack.  During  this 
time  the  child  should  be  placed  on  a  mild  diet ;  an  excess  of 
liquids  as  well  as  of  nitrogeneous  food  should  be  avoided. 
Small   amounts   of   meat,  fish,  and  eggs,  and  a  moderate 


1 84  THE   NERVOUS  DISEASES    OF   CHILDREN. 

amount  of  milk,  will  answer  the  purpose  better  than  if  the 
patient  is  placed  altogether  upon  nitrogeneous  or  upon  an 
excess  of  farinaceous  food. 

It  is  important  to  regulate  the  bowels  thoroughly,  and 
if  the  kidneys  are  not  very  active  to  increase  the  quantity 
of  urine  passed.  To  this  end  some  mild  alkaline  water, 
such  as  Vichy  or  Seltzer,  can  be  safely  recommended, 
either  alone  or  in  conjunction  with  milk. 

Innumerable  drugs  have  from  time  to  time  been  warmly 
recommended.  Some  years  ago  antipyrine  in  doses  of  five 
to  ten  grains,  according  to  the  age  of  the  patient,  was  quite 
in  vogue  ;  but  I  do  not  consider  it  promising  enough  to 
urge  its  use  in  the  face  of  the  well-known  risks  attending 
its  exhibition.  I  am  certain,  too,  that  phenacetine  in  five  to 
ten-grain  doses  has  little  or  no  effect.  I  have  seen  some  good 
results  from  the  combined  action  of  the  phenacetine  and 
caffeine  in  one-  to  three-grain  doses,  but  I  am  certain  that 
the  combination  is  not  in  any  way  superior  to  the  use  of 
caffeine  alone.  Caffeine  is  beyond  a  doubt  one  of  the  most 
serviceable  drugs  to  be  employed.  I  am  in  the  habit  of 
giving  it  to  children  under  fifteen  years  of  age  in  one-grain 
pills  or  powders,  which  are  to  be  repeated  every  twenty  min- 
utes until  distinct  relief  is  felt ;  and  after  the  first  relief  has 
been  procured  the  drug  should  be  continued  in  the  same 
dose  at  longer  intervals,  first  every  two,  then  every  three 
hours,  and  later  on,  for  a  period  of  about  a  week  after  the 
onset  of  the  attack,  it  can  be  given  safely  in  small  doses 
several  times  a  day,  according  to  the  condition  of  the 
child.  Caffeine  can  be  combined  with  iron,  with  quinine,  or 
with  arsenious  acid.  I  have  also  had  excellent  results  from 
a  combination  of  caffeine  and  cannabis  indica.  The  various 
preparations  of  the  latter  are  so  unreliable  that  it  is  as  well 
to  use  Herring's  extract  (gr.  -^  -  £).  A  caution  is  neces- 
sary, however,  as  regards  the  use  of  cannabis  indica,  for 
some  patients  are  peculiarly  intolerant  toward  this  drug, 
and  I  have  seen  distinct  symptoms  of  poisoning  from  small 
doses  repeatedly  employed.  While  the  drug  is  not  in 
reality  a  dangerous  one,  the  symptoms  due  to  the  admin- 
istration of  large  doses  are  so  disagreeable  and  are  so  apt 
to  alarm  the  patient  and  the  family  that  great  care  should 


MIGRAINE.  185 

be  exercised  in  the  use  of  the  drug;  but  if  the  fact  of  toler- 
ance on  the  part  of  the  patient  has  once  been  established 
no  trouble  is  apt  to  ensue  during  subsequent  trials.  Seguin 
thinks  that  the  drug  exerts  a  sedative,  even  paralyzing, 
influence  upon  the  third  cranial  nerve  and  its  attached 
muscles,  including  the  ciliary,  but  the  drug  has  as  good  an 
effect  in  cases  in  which  the  ocular  apparatus  has  been  de- 
termined to  be  entirely  normal. 

The  nitrite  of  amyl  has  been  suggested  on  physiological 
grounds  as  a  proper  remedy,  particularly  in  those  cases  in 
which  there  is  marked  pallor  of  the  face.  We  might  also 
expect  it  to  act  favorably,  as  it  does  in  epilepsy,  in  those 
cases  of  migraine  in  which  there  is  a  distint  sensory  aura. 
The  bromides  and  chloral  help  to  allay  the  nervous  excite- 
ment and  to  induce  sleep,  as  do  also  sulfonal,  trional,  vero- 
nal, and  chloralamid  in  four  to  fifteen-grain  doses;  but  I 
cannot  regard  them  as  in  any  sense  specific  remedies  in 
migraine.  Nitro-glycerine  (one  drop  of  the  one  per  cent, 
alcoholic  solution)  has  occasionally  helped  to  diminish  the 
severity  of  an  attack.  In  this  form  it  is  not  a  dangerous 
drug,  and  can  be  safely  administered  under  the  supervision 
of  a  physician,  of  a  nurse,  or  of  an  intelligent  mother.  The 
use  of  tea  anu  coffee  in  moderate  quantities  is  strongly  to 
be  recommended ;  I  have  known  children  suffering  from 
migraine  to  do  well  on  black  coffee  who  would  vomit  every- 
thing else  that  was  given.  I  feel  that  I  owe  much  to  Dr. 
Seguin's  suggestion  that  black  coffee,  without  sugar  and 
without  milk,  is  easily  digested,  and  that  the  general  preju- 
dice regarding  this  beverage  is  due  to  the  milk  and  sugar 
that  have  been  invariably  added,  and  that  have  given  rise 
to  fermentative  processes  in  the  gastro-intestinal  tract. 

As  for  the  correction  of  the  ocular  defects,  I  do  not  wish 
to  oppose  sensible  treatment  by  competent  oculists,  but  I 
do  oppose  treatment  based  upon  extreme  theories.  I  would 
have  the  eye  condition  corrected  on  the  principle  that  in 
migraine,  as  in  epilepsy,  every  physical  defect  should  be 
remedied;  but  I  must  insist  that  such  slight  errors  in  re- 
fraction and  in  accommodation  are  not  the  cause  of  this 
neurosis. 


CHAPTER  X. 

THE   DISORDERS   OF   SLEEP. 

The  healthy  new-born  child  sleeps  during  the  entire  day 
and  night,  except  when  it  is  being  nursed  or  dressed.  Dur- 
ing the  first  month  the  infant  is  awake  only  three  or  four 
hours  out  of  the  twenty-four,  falling  soundly  asleep  imme- 
diately after  nursing.  By  the  end  of  the  sixth  month  fifteen 
hours'  sleep  per  day  is  a  fair  average,  which  is  gradually  de- 
creased until  at  the  age  of  one  year  the  health)''  child  sleeps 
about  twelve  out  of  the  twenty-four  hours.  From  one  year 
to  four  years  an  average  of  ten  hours'  sleep  should  be  main- 
tained, and  up  to  the  age  of  ten  or  twelve  years  the  health  of 
the  child  demands  that  it  shall  sleep  quietly  for  at  least 
nine  hours  in  the  day.  If  there  is  a  marked  departure 
from  these  averages,  the  child  is  either  in  pain  or  some  of 
its  functions  are  not  entirely  normal.  In  the  acute  fevers 
or  in  any  febrile  condition  children  are  alternately  drowsy 
and  wakeful.  Disturbances  of  digestion  are  also  a  frequent 
cause  of  restless  sleep  or  of  prolonged  wakefulness. 

In  addition  to  these  special  conditions,  others  cause  in- 
somnia in  children  as  in  adults.  Poorly  ventilated  and  over- 
heated rooms  often  cause  restlessness,  and  for  this  reason  it 
is  important  that  a  window  should  be  kept  open  in  every 
bedroom  during  the  entire  night,  and  that  the  child  should 
be  covered  as  lightly  as  possible,  according  to  varying  at- 
mospheric conditions.  If  a  child  is  sleepless  in  the  absence 
of  unfavorable  conditions,  we  must  seek  some  other  cause 
for  the  insomnia.  In  many  cases  the  mother  or  nurse  has 
not  been  careful  to  engender  the  proper  habits  of  sleep. 
Infants  that  are  much  pampered,  that  are  taken  up  as  soon 
as  they  utter  the  first  cry,  or  that  are  wilfully  disturbed  by 
parents  or  nurse,  soon  become  poor  sleepers.  From  the 
first  week  of  life  the  child  should  be  laid  down  quietly  and 


THE    DISORDERS   OF  SLEEP.  1 87 

not  rocked  to  sleep,  and  should  not  be  disturbed  except  for 
good  reasons.  If  the  room  is  darkened  a  quiet  sleep  will 
come  on  very  naturally.  As  the  child  grows  older  it  is 
well  not  to  excite  it  just  before  the  hour  at  which  it  is  ex- 
pected  to  fall  asleep.  With  children  up  to  the  age  of  eight 
years  the  evening  meal  should  be  a  light  one,  and  their 
brains  should  not  be  disturbed  by  exciting  stories.  This 
should  be  observed  more  particularly  in  the  case  of  chil- 
dren who  exhibit  nervous  tendencies,  or  of  those  who  have 
passed  through  exhausting  diseases. 

The  normal  child  does  not  fall  asleep  unless  its  hunger 
has  been  appeased.  Insufficient  nourishment  is,  therefore, 
to  be  suspected  in  cases  of  persistent  wakefulness  in  young 
infants.  With  the  change  of  nurse,  or  after  weaning,  a 
restless  sleeper  will  often  develop  normal  habits  of  sleep. 
If  insomnia  continues  without  apparent  cause,  we  must  sus- 
pect some  graver  trouble.  In  the  earlier  stages  of  tubercu- 
lar meningitis,  in  tumor,  and  in  other  painful  diseases  of  the 
brain,  sleeplessness  is  often  one  of  the  earliest  symptoms. 

If  the  cause  of  insomnia  cannot  be  easily  made  out,  it 
is  well  to  fall  back  upon  faulty  methods  of  training.  Not 
very  long  since  a  child  of  five  years  was  brought  to  me  by 
its  mother,  who  stated  that  it  would  not  fall  asleep  if  it 
awoke  after  midnight.  The  mother  claimed  to  be  ignorant 
of  any  mistake  that  she  had  made  in  the  training  of  the 
child,  but  on  closer  questioning  I  heard  that  she  had  been 
in  the  habit,  whenever  her  husband  was  away,  of  taking  the 
child  out  of  its  bed  and  putting  it  in  her  own  bed.  After 
she  had  done  this  a  number  of  times  the  child  woke  up 
regularly  at  midnight^ and  would  cry  until  the  mother  took 
it  up.  The  mother,  a  very  nervous  woman,  excited  the 
child  by  her  caresses  and  her  despair  over  its  not  sleeping, 
and  thus  made  matters  worse  and  worse.  The  child  had 
been  sleeping  very  poorly  for  several  weeks  before  I  was 
consulted.  The  cure  was  a  very  simple  one.  The  child 
was  placed  under  the  care  of  a  sensible  trained  nurse,  who 
would  not  yield  to  its  solicitations,  and  after  a  few  nights 
of  restless  sleep  it  began  to  develop  proper  habits  and  soon 
slept  an  average  of  ten  hours. 

The  prognosis  is  invariably  favorable  unless  the  condi- 


1 88  THE  NERVOUS  DISEASES   OF  CHILDREN. 

tion  is  due  to  some  grave  disorder.  It  is  most  important 
to  insist  upon  absolute  regularity  in  putting  the  child  to 
bed  at  a  definite  hour  and  keeping  it  there  unless  it  is  neces- 
sary to  take  it  up.  If  this  should  prove  insufficient  a  warm 
bath  at  bedtime  will  be  conducive  to  sleep,  and  small  doses 
of  bromide  or  chloral  will  be  useful  to  engender  the  habit. 
Sulfonal  or  trional,  in  five-  to  ten-grain  doses,  veronal  in 
three-grain  doses,  may  be  substituted  for  the  bromide  or 
chloral  in  children  who  are  anaemic  or  poorly  nourished. 
All  hypnotics  should  be  withdrawn  as  soon  as  possible,  for 
there  is  great  danger  of  accustoming  the  child  to  these 
drugs.  In  some  cases  the  mere  regulation  of  the  meals,  and 
the  avoidance  of  all  indigestible  articles  of  diet,  will  be  suffi- 
cient to  restore  sleep.  In  children  who  are  impressionable 
it  is  of  the  greatest  importance  to  avoid  telling  them  stories 
or  even  engaging  them  in  play  in  the  latter  part  of  the  day. 

Pavor  NOCTURNUS,  or  night  fear,  is  a  condition  which 
is  often  troublesome  both  to  the  parents  and  the  physician. 
Children  affected  with  this  disturbance  wake  up  a  few 
hours  after  they  have  entered  into  a  sound  sleep,  are  pos- 
sessed as  a  rule  by  great  fear,  fail  to  make  out  their  sur- 
roundings, and  act  as  though  in  a  temporarily  dazed  con- 
dition. They  do  not  understand  the  soothing  words  of 
parent  or  physician,  and  often  continue  in  a  state  of  excite- 
ment until  they  return  to  full  consciousness  or  are  over- 
come by  sleep. 

There  seems  to  be  no  good  reason  to  give  any  other  in- 
terpretation of  this  condition  than  that  the  child  has  had  a 
horrible  dream,  and  that  the  substance  of  the  dream  is  con- 
tinued in  the  half-waking  state,  or  that  the  child  is  possessed 
by  fear,  which  is  the  natural  result  of  the  dream  and  can- 
not shake  off  the  fear  until  it  fully  realizes,  if  old  enough, 
that  its  experiences  have  been  unreal. 

This  condition  may  occur  in  any  child,  but  it  is  decidedly 
more  frequent  in  children  who  have  shown  previous  nervous 
symptoms,  or  in  children  of  highly  neurotic  parents.  It  is 
not  infrequent  in  those  who  are  pressed  by  school  duties, 
and  who  go  to  bed  with  a  fear  that  their  lessons  have  not 
been  properly  done  for  the  next  day.  This  knowledge  is 
sufficient  to  prevent  sound  sleep,  and  during  the  hours  of 


THE   DISORDERS   OF  SLEEP.  189 

restlessness  vivid  dreams  are  very  apt  to  occur.  It  was 
formerly  supposed  that  late  and  indigestible  meals  were 
the  chief  cause  of  these  noctural  disturbances.  Inasmuch 
as  any  indigestion  may  give  rise  to  restless  sleep,  this  factor 
may  have  to  be  taken  into  account ;  but  over-work,  over- 
excitement,  the  reading  of  horrible  or  fascinating  tales  just 
before  bedtime,  constitute  a  much  more  important  etiologi- 
cal factor. 

These  noctural  attacks  often  occur  a  single  time,  but 
with  most  children  are  frequently  repeated  during  the  ear- 
lier years  of  life,  say  between  the  ages  of  three  and  eight 
years.  With  the  growth  of  the  child  and  the  improvement 
of  its  nervous  system  the  attacks  cease. 

The  prognosis  of  this  condition  is  entirely  favorable  ; 
though  I  can  recall  one  case  of  a  girl,  aged  six  years,  the  child 
of  an  hysterical  mother,  in  whom  the  fright  connected  with 
one  of  these  nocturnal  attacks  was  the  cause,  or  at  least  the 
first  beginning,  of  a  severe  chorea,  which  lasted  for  many 
months.  In  another  child,  about  nine  years  of  age,  who  had 
had  attacks  of  epilepsy  ever  since  early  childhood,  and  who 
was  particularly  subject  to  nocturnal  attacks  of  epilepsy, 
the  epileptic  attacks  ceased  upon  bromide  treatment,  but 
every  now  and  then  the  child  would  have  a  very  marked 
spell  of  pavor  nocturnus,  which  would  disturb  her  almost 
as  much  as  an  epileptic  seizure  would. 

Conditions  similar  to  those  of  pavor  nocturnus  occur  at 
times  during  the  day.  A  little  patient  of  mine,  aged  five 
years,  would,  in  a  very  unaccountable  manner,  while  walk- 
ing with  her  mother  on  the  street,  suddenly  hide  her  face  in 
the  folds  of  the  mother's  dress,  and  bury  her  head  for  some 
time,  in  order  (as  she  explained  later  on),  not  to  see  the  hor- 
rible black  things  coming  toward  her.  This  child  showed 
no  other  symptom  of  a  nervous  disposition,  but  was  for  a 
time  in  great  dread  of  the  recurrence  of  these  experiences. 
That  they  were  the  result  of  visual  hallucinations  during 
the  waking  hours,  I  can  hardly  doubt.  They  disappeared 
entirely  under  sedative  treatment,  which  was  given  carefully 
for  some  months. 

In  regard  to  treatment,  the  most  important  point  is  to  ex- 
ercise every  possible  precaution  in  order  that  unnecessary 


I90  THE  NERVOUS  DISEASES   OF   CHILDREN. 

nervous  excitement,  late  in  the  day,  may  be  avoided,,  I 
prohibit  all  school-work  after  five  in  the  evening  ;  do  not 
allow  the  child  to  read  or  be  read  to  after  this  hour,  and  in- 
sist on  a  very  light  meal  in  the  evening.  After  this  meal 
the  child  is  to  remain  awake  for  at  least  an  hour,  and  may 
indulge  in  some  simple  game,  or  is  allowed  to  frolic  about 
in  a  quiet  manner.  Small  doses  of  the  bromides,  from  five 
to  ten  grains,  according  to  the  age  of  the  child,  about  an 
hour  before  bedtime  will  be  the  best  remedy.  If  neces- 
sary, small  doses  of  chloral  may  be  given  with  the  bromides. 
If  the  attacks  return  very  frequently  I  have  found  it  more 
satisfactory  to  give  five-  or  ten-grain  doses  of  chloralamid, 
or  of  trional,  every  night,  or  every  second  or  third  night, 
until  all  excitement  has  subsided. 

Enuresis  Nocturna  is  another  very  common  form  of 
disturbed  sleep.  Children  who  have  been  well  trained, 
and  who  have  learned  to  observe  all  the  habits  of  cleanli- 
ness during  the  day,  are  frequently  disturbed  by  involuntary 
micturition  during  the  night.  This  is  most  apt  to  occur 
during  the  earlier  hours  of  sleep,  when  sleep  is  most  pro- 
found. The  soundness  of  sleep  alone  may  be  a  sufficient 
cause  for  this  phenomenon,  inasmuch  as  children  do  not 
perceive  in  sleep  those  symptoms  of  vesical  irritation  which 
prompt  them  to  evacuate  the  bladder  during  waking  hours, 
and  during  light  sleep.  In  others,  and  possibly  in  the  ma- 
jority of  cases,  it  is  due  to  indifference  on  the  part  of  the 
child,  or  to  an  aversion  to  allow  its  sleep  to  be  interrupted. 
In  some  few  cases  enuresis  is  the  result  of  frightful  dreams, 
the  sudden  impulse  to  evacuate  the  bladder  accompanying 
such  a  dream  as  it  would  any  emotional  condition  during 
wakefulness.  If  due  to  this  latter  cause  the  enuresis  is  not 
frequently  repeated.  Enuresis  has  been  observed  to  be  a 
symptom  of  nocturnal  epilepsy.  If  so,  it  occurs  at  rare  in- 
tervals ;  the  child  is  entirely  unconscious  of  it,  and  often 
shows  some  other  symptom,  such  as  drowsiness  and  head- 
ache on  the  following  morning. 

The  condition  is  a  very  stubborn  one,  and  all  possible 
methods  of  treatment  have  been  suggested.  I  am  con- 
vinced that  the  cure  of  the  condition  is  dependent  chiefly 
upon   careful   training.     The   necessity  of   evacuating   the 


THE   DISORDERS   OE  SLEEP.  \'jl 

bladder  should  be  impressed  upon  children  after  the  age 
of  two  years.  The  child  should  be  made  to  void  urine  im- 
mediately before  going  to  bed,  and  should  be  aroused  one 
and  a  half  to  two  hours  after  it  has  fallen  asleep,  and  should 
be  induced  to  pass  water  again.  By  giving  relatively  few 
liquids  after  four  or  five  o'clock  in  the  afternoon,  I  have 
found  that  the  tendency  to  enuresis  is  often  checked. 

If  these  simple  measures  are  not  sufficient,  a  few  drops 
of  the  tincture  of  belladonna,  given  at  bedtime,  will  be  use- 
ful, and  if  the  condition  is  due  to  frightful  dreams,  or  to 
cerebral  excitement  continued  during  sleep,  small  doses  of 
bromides  may  be  given.  \  Many  medical  men  have  seriously 
suggested  that  corporal  punishment  applied  to  the  nates  is 
the  only  efficient  remedy,  and  some  have  gone  so  far  as  to 
suggest  that  the  cutaneous  hyperemia  caused  by  such  pun- 
ishment explains  the  relief  afforded.  This  explanation  is 
not  satisfactory  ;  but  whatever  the  mode  of  action  may  be, 
it  is  very  certain  that  such  punishment  need  not  be  inflicted 
in  the  majority  of  cases,  and  that  the  desired  end  can  be 
attained  by  other  methods  of  training. 

Somnambulism  is  by  no  means  rare  in  children,  and  occurs  most  fre- 
quently in  those  who  are  of  a  nervous  temperament,  or  who  have  passed 
through  some  severe  excitement.  I  have  not  known  them  to  perform 
any  of  the  marvellous  tricks  which  are  generally  accredited  to  somnambulists, 
such  as  climbing  out  of  windows  and  on  the  roof,  and  the  like  ;  but  they  are 
apt  enough  to  walk  through  the  house,  from  one  room  to  another,  and  to  go 
through  a  number  of  purposive  actions  without  being  at  all  conscious  of  what 
they  are  doing.  Nightmare  is  evidently  closely  related  to  this  condition  of 
somnambulism,  and  is  generally  associated  with  horrible  dreams,  from 
which  the  child  awakens  badly  frightened,  but  is  quieted  much  more  rap- 
idly than  in  the  condition  which  is  described  as  pavor  nocturnus.  Night- 
mare and  somnambulism  occur,  as  a  rule,  at  rare  intervals  and  can  generally 
be  traced  to  some  emotional  excitement,  or  to  some  acute  indigestion.  The 
treatment  of  these  conditions  is  exactly  the  same  as  that  of  night-terror. 


CHAPTER   XL 

VASOMOTOR   AND   TROPHO-NEUROSES. 

In  this  chapter  a  brief  description  will  be  given  of  a  num- 
ber of  rare  and  peculiar  diseases,  which  must  for  the  pres- 
ent be  classified  under  the  rather  vague  title  of  vasomotor 
and  tropho-neuroses.  Grouping  them  together  under  one 
head  is  simply  a  matter  of  convenience. 

EXOPHTHALMIC    GOITRE. 

This  disease,  which  is  generallv  termed  Graves's  disease 
by  the  English  writers,  and  Basedow's  disease  by  the  Ger- 
mans, occurs  chiefly  in  adult  life,  yet  a  sufficient  number  of 
cases  begin  during  childhood  to  make  it  incumbent  upon 
us  to  give  a  brief  description  of  the  disorder. 

The  cardinal  symptoms  are  : 

i.  Excessive  cardiac  action.  This  is  by  far  the  most 
constant  symptom  of  all,  and  is  often  present  in  the  earlier 
stages  of  the  disease  when  the  other  two  cardinal  symptoms 
— swelling  of  the  thyroid  and  the  protrusion  of  the  eyes — 
are  not  yet  in  evidence.  The  pulse-rate  may  vary  between 
90  and  200.  In  the  later  stages  of  the  disease  the  left  ventri- 
cle, and  rarely  the  right  ventricle,  may  become  hypertro- 
phied  and  dilated.  The  heart-sounds  are  clear  but  unusu- 
ally loud.  All  the  arteries  pulsate  very  distinctly,  so  that 
the  pulse  of  the  abdominal  aorta  may  possibly  be  observed 
through  the  abdominal  walls,  and  the  pulse  in  the  carotids 
may  be  so  strong  as  to  cause  distinct  pulse-like  vibrations 
of  the  entire  head.  The  pulse  may  even  be  noticeable  in 
the  retinal  arteries,  and  a  further  disturbance  of  the  entire 
vascular  system  may  be  noted  by  a  dilatation  of  the  capil- 
laries in  the  skin,  by  the  presence  of  distinct  tdclies  cere- 
brates, and  a  dilatation  of  all  the  larger  superficial  veins.     If 


VASOMOTOR  AND  TROPHO-NEUROSI  193 

venous  stasis  is  marked,  oedema  may  occur  in  various  parts 
of  the  body.  It  is  also  owing  to  the  increased  tension  that 
hemorrhages  are  frequent  from  the  nose,  and  even  in  the 
stomach  and  bowels.  Cerebral  hemorrhages  have  also  been 
described  in  Graves's  disease.  A  particularly  characteristic 
symptom,  which  is  found  in  exophthalmic  goitre,  and  not 
in  any  form  of  enlargement  of  the  thyroid,  is  the  whirring 
felt  if  the  finger  is  placed  upon  the  goitre. 

2.  The  second  cardinal  symptom  is  the  enlargement  of 
the  thyroid  gland.  It  is  present  in  the  vast  majority  of 
the  cases,  though  a  number  of  authors  have  taken  pains  to 
describe  cases  of  this  vasomotor  disease  in  which  the 
thyroid  was  not  enlarged  ;  but  the  enlargement,  as  a  rule, 
comes  on  some  time  after  the  tachycardia.  The  gland,  after 
it  has  once  become  enlarged,  increases  rapidly,  and  may  give 
rise  to  a  great  deal  of  inconvenience.  The  enlarged  gland, 
or  struma,  is  extremely  vascular,  and  the  tissue  of  the  gland 
is  not  only  hyperasmic  but  very  soon  enters  into  a  state  of 
hyperplasia  with  a  tendency  to  a  fibrinous  degeneration. 
In  keeping  with  these  changes  the  goitre  may  in  the  begin- 
ning be  soft  and  yielding,  later  on  it  will  become  hard  to  the 
touch.  The  enlargement  of  the  thyroid  is,  as  a  rule,  bilateral, 
but  generally  asymmetrical.  The  right  half  is  more  fre- 
quently enlarged  than  the  left  half. 

3.  The  third  cardinal  symptom  is  exophthalmus,  or  pro- 
trusion of  the  eyes.  This  is  the  least  constant  of  the  three 
symptoms.  If  present  it  is  generally  bilateral,  but  in  some 
instances  one  eye  only  may  be  affected,  or  the  protrusion  of 
one  may  be  very  much  greater  than  that  of  the  other  eye. 
The  causes  of  this  protrusion  have  been  in  dispute,  but  it  is 
more  than  probable  that  the  interference  with  the  venous 
current,  as  well  as  the  arterial  congestion,  are  the  prime 
causes  of  this  protrusion.  The  great  variability  of  this 
symptom  during  life,  its  disappearance  almost  immediately 
after  death,  and  the  pulsation  of  the  vessels  in  the  fundus, 
lend  support  to  this  view.  It  can  hardly  be  doubted,  how- 
ever, that  in  some  cases  the  development  of  fat  and  of  cellu- 
lar tissue  in  the  retro-bulbar  space  may  make  the  protrusion 
still  more  marked. 

Vision  is  not  affected  in  spite  of  the  exophthalmus,  and 


194  THE  NERVOUS  DISEASES   OF   CHILDREN. 

the  only  changes  observed  were  those  recorded  by  Kast 
and  Willbrand,  who  have  noted  a  limitation  of  the  field  of 
vision  in  this  disease  without  any  evidence  of  hysteria.  The 
pupils  may  be  unequal,  but  react  promptly.  Ulcerations  of 
the  cornea,  occurring  in  consequence  of  insufficient  protec- 
tion of  the  bulb,  have  been  noted ;  as  well  as  some  interfer- 
ence with  the  lachrymal  secretion. 

Among  the  accessory  symptoms  those  relating  to  the 
movements  of  the  eyes  are  the  most  important.  Graefe's 
symptom  refers  to  defective  movements  of  the  upper  eye- 
lid on  vision  downward.  The  upper  lid  does  not  follow 
promptly  the  downward  movement  of  the  bulb,  and  the  con- 
junctiva remains  visible  between  the  pupil  and  the  margin 
of  the  lid.  This  symptom  evidently  bears  some  relation  to 
the  phenomenon  described  by  Stellwag  consisting  of  a  dila- 
tation of  the  palpebral  fissure.  Graefe's  symptom,  as  far  as 
can  be  ascertained,  is  caused  neither  by  the  protrusion  of 
the  eyes  nor  by  a  spasm  of  the  levator  or  a  paresis  of  the 
orbicularis,  but  is  explained  most  readily  as  the  result  of 
Stellwag's  phenomenon.  Moebius  has  described  another 
symptom  implying  a  deficiency  in  the  power  of  accommo- 
dation. There  is  defective  convergence  of  the  axes  of  the 
eyes.  These  three  symptoms  are  not  absolutely  pathogno- 
monic of  Basedow's  disease,  as  each  one  has  been  described 
in  connection  with  other  neuroses ;  but  if  they  are  associated 
with  either  one  of  the  cardinal  symptoms  they  help  to  cor- 
roborate the  diagnosis  of  Basedow's  disease. 

The  next  most  frequent,  and  perhaps  most  important, 
symptom  is  a  tendency  to  profuse  diarrhoea.  This  is  en- 
tirely independent  of  any  gastro-intestinal  disease,  often 
resists  treatment,  and  adds  greatly  to  the  patient's  discom- 
fort and  to  the  general  depreciation  of  his  health.  Gastric 
crises  have  been  described  in  some  cases,  and  frequent 
vomiting  is  not  an  unusual  symptom.  Most  patients  exhibit 
considerable  dyspnoea,  resembling  true  asthma,  and  Louise 
Bryson  has  referred  to  defective  expansion  of  the  chest  on 
inspiration.  Glycosuria  and  albuminuria  have  been  re- 
ported ;  the  former,  according  to  the  investigations  of  Chvo- 
stek,  is  of  the  alimentary  order.  In  some  few  instances, 
however,  diabetes  mellitus  has  occurred  as  a  complication 


VASOMOTOR  AND   TROPIIO-NEUROSES.  ig$ 

of  Basedow's  disease.  This  association  is  not  a  strange  one, 
since  both  diseases  are  often  developed  after  severe  emo- 
tional excitement.  Of  the  disturbances  in  menstruation  we 
need  take  no  account,  as  we  are,  after  all,  concerned  with 
the  disease  as  it  occurs  in  children. 

Trophic  and  vasomotor  disturbances  of  the  skin  are  fre- 
quent. Pigmentation,  leukoderma,  loss  of  hair,  premature 
grayness  of  hair,  and  even  scleroderma,  as  well  as  herpes, 
have  been  observed.  A  very  remarkable  symptom  of 
Graves's  disease  is  a  tendency  to  profuse  sweating.  This 
may  be  universal  or  partial,  sometimes  strictly  unilateral. 
This  tendency  must  be  held  to  account  for  the  remarkable 
diminution  in  electrical  resistance  which  has  been  observed 
in  the  skin  of  patients  suffering  from  this  disease.  This  fact 
was  first  insisted  upon  by  Vigouroux,  and  later  by  Eulen- 
burg.  Kahler  and  others  have  shown  that  this  reduction  in 
resistance  to  the  galvanic  current  is  due  to  the  fact  that  the 
relative  minimum  of  resistance  is  reached  much  more  easily 
in  patients  suffering  from  Graves's  disease  than  in  healthy 
individuals.  Kahler  has  also  shown  that  this  diminished 
resistance  is  not  due  to  an  hyperasmia,  as  was  supposed  by 
some,  but  it  is  due  to  excessive  moisture  of  the  skin.  A 
faint  tremor  is  frequently  observed,  and  by  some  is  con- 
sidered to  be  one  of  the  cardinal  symptoms,  but  it  is  not  as 
constant  as  the  three  cardinal  symptoms  we  have  mentioned. 
The  tremor  is  rhythmical  and  consists  of  eight  or  nine  vibra- 
tions per  second.  Choreiform  tremor  occasionally  occurs, 
and  true  chorea  and  epilepsy  are  complications  met  with 
in  a  certain  number  of  cases.  These  complications  are  in 
all  probability  purely  accidental,  and  cannot  readily  be  ex- 
plained on  the  supposition  that  the  vasomotor  changes  in 
Basedow's  disease  are  the  cause  of  the  chorea  and  epi- 
lepsy. 

In  addition  to  the  preceding  symptoms  mental  changes 
deserve  some  notice,  and  among  these  we  may  note  irrita- 
bility of  temper,  sudden  changes  of  temperament,  and  the 
occasional  occurrence  of  mania  or  melancholy.  Some  of 
these  psychic  changes  are  developed  in  the  earlier  stages  of 
the  disease,  others  are  later,  and  possibly  accidental  compli- 
cations.     If  we  add  anaemia,  albuminuria,  and   a  general 


I96  THE   NERVOUS  DISEASES    OF    CHILDREN. 

cachexia,  we  have  completed  the  list  of  the  symptoms  typi- 
cal of  Graves's  disease. 

The  morbid  anatomy  of  Graves's  disease  has  been  the 
subject  of  much  discussion.  The  cervical  sympathetic  has 
been  given  a  very  important  part.  A  number  of  authors 
have  reported  actual  changes  in  the  cervical  ganglia  as  the 
result,  of  connective-tissue  proliferation,  and  others  have  re- 
ported atrophy  and  degeneration  of  the  nerve-elements  ;  but 
since  Hale  White  has  proven  that  similar  changes  occur  in 
persons  who  have  not  exhibited  the  symptoms  of  Graves's 
disease,  and  that  in  persons  who  have  died  from  this  disease 
such  changes  have  not  been  found,  the  force  of  all  these 
findings  has  been  much  diminished.  The  same  may  be  said 
of  hemorrhages  into  the  fourth  ventricle,  or  atrophy  of  the 
corpus  restiforme,  to  which  Mendel  and  Leube  have  attached 
some  importance.  The  heart  has  naturally  exhibited  hyper- 
trophy or  dilatation,  insufficiency  of  the  mitral  valve,  and 
symptoms  of  mild  endocarditis,  but  these  changes  are 
clearly  secondary  and  do  not  at  least  hold  any  causal  rela- 
tion to  the  disease. 

The  theories  regarding  the  pathology  of  the  disease 
have  been  still  more  conflicting.  Basedow  supposed  the 
disease  due  to  chlorotic  changes  in  the  blood  ;  but  this  ex- 
planation is  evidently  insufficient,  as  the  disease  frequently 
affects  persons  in  robust  health  who  are  far  from  anaemic. 
The  close  resemblance  between  the  symptoms  following 
upon  section  of  the  cervical  sympathetic  and  those  of 
Graves's  disease  has  led  many  writers  to  attribute  exophthal- 
mic goitre  to  disease  of  the  sympathetic  !  Section  of  the 
sympathetic  produces  irritation,  and  the  symptoms  follow- 
ing the  experiment  upon  animals  are  clearly  the  result  of 
irritation ;  but  we  cannot  suppose  any  condition  of  irritation 
to  last  for  years,  and  must  therefore  seek  some  other  expla- 
nation. Friedreich  was  inclined  to  the  theory  that  dilatation 
of  the  vessels  is  the  result  of  paralysis  of  the  sympathetic ; 
that  dilatation  of  the  coronary  arteries  caused  tachycardia, 
and  that  this  increased  flow  of  blood  to  the  cardiac  muscle 
produced  an  increased  activity  of  the  heart,  and  that  all  the 
other  symptoms  of  Graves's  disease,  the  exophthalmus  and 
the  thyroid  swelling,  were  the  result  of  the  arterial  hyper- 


VASOMOTOR  AND   TROPHO-NEUROSES.  1 97 

asmia.  Granting  that  the  cardinal  symptoms  can  be  ex- 
plained in  this  way,  the  many  accessory  symptoms  cannot 
be  accounted  for  on  this  theory.  Other  authors  have  in- 
sisted on  the  bulbar  origin  of  Graves's  disease,  and  have  sup- 
posed that  a  lesion  involving  the  nuclei  of  the  vagus  was 
sufficient  to  account  for  all  the  symptoms,  and  some  post- 
mortem findings  have  given  support  to  this  view. 

A  number  of  authors,  more  especially  Moebius,  have  in- 
sisted on  the  probability  of  the  toxic  origin  of  the  disease,  and 
have  supposed  that  by  the  increased  action  of  the  thyroid 
gland  a  toxine  is  produced  which  is  primarily  responsible 
for  all  the  symptoms.  In  a  recent  and  very  able  review  of  all 
the  conflicting  theories  (haematogenic,  neurogenic  and  thyre- 
ogenic),  Eulenburg  (Die  Deutsche  Klinik,  1904)  concludes 
that  there  must  be  qualitative  as  well  as  quantitative 
changes  in  the  secretion  of  the  thyroid  gland  to  account  for 
the  symptoms  of  Basedow's  disease.  These  quantitative 
changes,  Eulenburg  supposes,  are  due  to  an  abnormal  nerve 
influence,  the  exact  nature  of  which  is  still  to  be  determined. 

The  course  of  the  disease  is,  as  a  rule,  extremely  chronic. 
The  onset  may  be  very  sudden,  and  even  violent,  but  after  a 
while  the  symptoms  subside  in  severity  and  remain  con- 
stant for  a  long  period  of  years ;  but  the  prognosis  is  not 
necessarily  as  grave  as  it  is  generally  supposed  to  be.  I 
have  myself  seen  a  number  of  cases  of  very  marked  im- 
provement and  of  total  disappearance  of  all  the  symptoms. 
If  death  ensues  it  is,  as  a  rule,  due  to  some  complicating 
disease,  or  to  extreme  exhaustion  from  diarrhoea,  or  from 
cardiac  weakness,  or  possibly  from  acute  mania. 

Treatment. — Absolute  rest  and  careful  feeding  are  the 
most  important  measures.  The  recoveries  which  I  have 
seen  have  been  in  patients  who  have  been  placed  on  the 
rest-cure.  The  entire  freedom  from  excitement,  and  regular 
feeding  have  tended  to  lessen  all  the  nervous  symptoms  and 
have  influenced  the  diarrhoeas  which  are  often  the  most  ex- 
asperating and  exhausting  symptom.  The  tachycardia  is 
remarkably  lessened  and  the  dyspnoea  is  naturally  benefited 
by  rest  in  bed.  The  diet  should  be  simple.  It  is  well  to 
avoid  stimulants  and  excitants  as  well  as  carbonated  waters. 
The  excessive  dilatation  of  the  stomach,  resulting  from  the 


I98  THE  NERVOUS   DISEASES   OF   CHILDREN. 

last-named,  acts  unfavorably  upon  the  heart.  Hydrothera- 
peutic  procedures,  consisting  of  cold  baths  followed  by 
massage,  of  douches  and  drip  sheets,  have  been  recom- 
mended by  many.  Mineral  baths  may  have  some  effect 
upon  the  constitutional  symptoms  and  thus  favorably  in- 
fluence the  disease.  Electricity  has  been  warmly  recom- 
mended by  many,  with  special  reference  to  the  part  played 
by  the  cervical  sympathetic  and  vagus.  To  give  this 
method  a  fair  trial  it  is  best  to  place  the  anode  by  turns 
over  the  goitre,  the  heart,  and  over  the  abdomen.  The  cur- 
rent should  be  mild  and  continuous.  Recently  Rockwell 
has  recommended  the  use  of  the  faradic  current. 

As  far  as  medicinal  treatment  is  concerned  the  drugs  in- 
fluencing the  heart's  action  have  been  most  in  favor ;  above 
all,  digitalis  and  strophanthus.  While  these  drugs  act 
favorably  in  some  cases  they  unquestionably  exert  an  un- 
favorable influence  in  others.  I  have  found  that  exces- 
sive palpitation  of  the  heart  could  be  controlled  very  much 
better  by  cold  applications  to  the  cardiac  region,  and  by 
the  use  of  the  galvanic  current,  with  the  anode  over  the 
heart.  I  am  willing,  however,  to  concede  that  the  effect  of 
the  latter  may  be  due  to  suggestion.  If  there  is  great 
emotional  excitement  bromides  in  small  doses  may  be  given 
with  advantage.  Nitrite  of  amyl  and  nitro-glycerine  have 
been  recommended,  but  they  are  to  be  used  with  great  cau- 
tion, especially  in  children,  and  the  result  is  a  doubtful  one 
at  best.  Ergot  has  been  administered  in  some  instances, 
and  the  subcutaneous  injections  of  ergotin  into  the  goitre 
have  been  practised,  but  the  results  have  not  been  favorable 
enough  to  warrant  its  continuance. 

Thyroidectomy,  or  excision  of  a  part  of  the  enlarged 
gland,  has  come  into  vogue,  and  has  been  reported  by 
many  German,  English,  and  American  writers.  Improve- 
ment following  the  operation  has  been  observed,  and  in  my 
opinion  is  sufficiently  marked  to  warrant  further  trial  of 
this  procedure  ;  but  I  am  not  aware  that  the  operation  has, 
as  yet,  been  attempted  in  children.  Sufficient  time  has  not 
yet  elapsed  to  decide  whether  the  relief  obtained  by  the 
operation  is  merely  temporary,  or  whether  a  permanent 
cure  has  been  effected.     If  the  enlarged  gland  presses  upon 


VASOMOTOR  AND   TROPIIO-NEUROSES.  [99 

the  trachea  and  menaces  life,  the  operation  is  surely  justi- 
fied. Moebius,  Leyden,  and  others  have  advised  the  use  of 
Anti-thyroidin,  Rodagen  (prepared  from  the  milk  of  goats 
whose  thryoid  glands  had  been  removed) ;  but  the  effect  of 
these  newer  remedies  is  still  sub  judice — and  great  caution 
should  be  exercised  in  administering  them. 

Thyroid  Enlargements  at  the  Age  of  Puberty. — At  this  period 
the  enlargement  is  often  developed  quite  suddenly,  and  is  so  marked  as  to 
arouse  suspicion  of  incipient  exophthalmic  goitre.  As  such  girls  are  often 
anaemic,  and  have  a  high  pulse-rate  the  suspicion  of  serious  disease  becomes 
still  stronger ;  but  such  enlargement  of  the  thyroid  gland  does  not  continue 
for  any  great  length  of  time,  and  none  of  the  other  important  symptoms  of 
Graves's  disease  are  developed. 

Tachycardia  should  also  be  mentioned,  as  it  may  occasionally  give  rise 
to  the  suspicion  of  Graves's  disease.  An  excessively  high  pulse-rate  is  fre- 
quent in  children,  but  true  tachycardia  in  children  has,  to  my  knowledge, 
rarely  been  reported.  I  have  seen  several  interesting  cases  of  this  kind,  and 
one  of  them  was  of  unusual  interest.  It  was  the  case  of  a  boy,  of  twelve 
years  of  age,  who  had  been  under  my  treatment  for  infantile  cerebral  hemi- 
plegia, and  had  sufficiently  recovered  from  this  condition  to  be  able  to  go 
about  freely,  to  attend  school,  and  to  play  with  boys  of  his  age.  While 
playing  on  a  cold  winter's  day  he  was  struck  in  the  neck  by  an  icy  snow- 
ball. Directly  he  felt  faint,  but  did  not  lose  consciousness.  As  soon  as  he 
was  put  to  bed  the  tachycardia  became  evident,  and  his  family  physician  was 
sent  for.  From  the  first  day  until  about  three  weeks  after  the  accident  the 
pulse  was  at  no  time  less  than  200  per  minute,  and  often  exceeded  this  rate, 
so  that  the  number  could  not  well  be  counted.  None  of  the  drugs  ex- 
hibited—such as  bromides,  strophanthus,  digitalis,  aconitia — had  the  slightest 
effect  upon  the  heart ;  but  after  a  period  of  about  three  weeks  the  symptoms 
subsided  quite  suddenly,  and  the  boy,  now  a  man,  has  been  entirely  well  for 
many  years.  The  injury  to  the  cervical  region,  and  possibly  to  the  cervical 
sympathetic,  causing  tachycardia,  is  of  interest,  if  we  recall  the  part  that  the 
cervical  sympathetic  is  supposed  by  many  to  play  in  the  causation  of  Graves's 
disease.  The  treatment  of  such  cases  should  consist  of  rest,  and  the  appli- 
cation of  an  ice-bag  to  the  heart  and  to  the  cervical  spine ;  bromides,  heart 
tonics,  and  aconitia  may  be  tried  according  to  the  indications  of  the  case. 

myxcedema. 

Myxcedema  is  a  form  of  trophic  disease  to  which  attention  should  be 
directed.  The  disease  is  of  unusual  interest,  but  we  need  not  go  into  a  full 
discussion  of  the  subject,  as  the  one  form  of  it  which  occurs  in  children — 
myxcedematous  idiocy — will  be  referred  to  in  the  Chapter  on  Idiocy.  Myx- 
cedema was  first  fully  described  by  Gull,  in  1873;  four  years  later  similar 
cases  were  reported  by  Ord,  and  since  that  time  innumerable  authors  in 


200 


THE   NERVOUS  DISEASES    OF   CHILDREN. 


England,  America,  France,  and  Germany  have  studied  this  trophoneurosis. 
Special  interest  has  been  attached  to  this  disease,  since  Kocher  described  a 
condition  following  upon  removal  of  the  thyroid,  resembling  myxcedema,  to 
which  he  gave  the  name,  cachexia  strumipriva.  The  Commission  that  re- 
ported to  the  Clinical  Society  of  London,  in  1888,  agreed  that  myxcedema, 
cachexia  strumipriva,  sporadic  cretinism,  represented  one  and  the  same  mor- 
bid entity.      The  condition  following  upon  removal  of  the  thyroid  gland, 

the  results  of  physiological  ex- 
periments (implantation  of  the 
glands  into  the  abdomen  of 
animals  —  Schiff,  Horsley,  and 
others),  and  above  all,  the  brill- 
iant achievements  of  thyroid 
feeding,  have  proved  conclu- 
sively that  deficient  action  of 
the  thyroid  gland  is  the  most 
important  factor  in  the  etiology 
of  myxcedema. 

Sy7nptoms. — Of  the  symp- 
toms of  myxcedematous  idiocy 
(the  congenital  form)  we  need 
here  merely  state  that  the  skin 
is  glossy  or  hard  ;  the  lips  and 
tongue  are  thick  and  large  ;  the 
hair  is  stiff  and  dry ;  the  child 
is  dwarfish  in  stature  ;  the  men- 
tal condition  is  that  of  marked 
idiocy  or  of  imbecility.  There 
is  always  absence  of  the  thyroid 
gland. 

Myxcedema  resembling  that 
of  the  adult  may  come  on  at 
the  age  of  puberty,  or  even  ear- 
lier. It  is  characterized  by  the 
following  symptoms :  A  gen- 
eral increase  in  the  bulk  of  the 
body ;  the  skin  is  firm  and  in- 
elastic, does  not  pit  upon  press- 
ure, and  is  dry  and  rough  ;  the 
folds  of  the  skin  disappear,  and  there  is  a  general  obliteration  of  all  the  lines 
of  the  skin,  particularly  in  the  face,  giving,  as  a  rule,  an  older  and  more  stu- 
pid expression  to  the  face  ;  the  nostrils  and  lips  are  very  much  enlarged. 
There  is  distinct  apathy  and  slowness  of  speech,  as  well  as  of  action,  in  some 
instances  ;  the  mental  changes  include  delusions  which  lead  to  dementia. 
Albuminuria  and  glycosuria  have  been  observed  ;  but  these  are  accidental 
complications,  no  doubt. 

The  disease  cannot  be  mistaken  for  any  other,  except  possibly  chronic 


Fig.  52. — Case  of  Myxcedema  with  Idiocy.    Pa- 
tient Twelve  Years  Old :  Dwarfish  in  Stature. 


VASOMOTOR  AND   TROPHO-NEUROSES.  201 

nephritis ;  but  the  lack  of  pitting,  the  examination  of  the  urine,  and  the  pe- 
culiar expression  of  the  face  will  help  to  remove  all  doubts. 

The  disease  is  slowly  progressive,  and  may  last  for  ten,  fifteen,  or  more 
years.  The  prognosis  was  hopeless  until  the  recent  discovery  of  thyroid 
feeding,  and  success  of  this  warrants  us  in  rejecting  for  the  present  every  other 
form  of  treatment. 

Treatment  is  to  consist  of  the  administration  of  the  pulverized  thyroid 
gland  of  the  sheep,  which  is  to  be  had  in  all  countries.  Armour's  prepara- 
tion has,  in  my  experience,  been  the  most  satisfactory.  Patients  taking  thy- 
roid gland  should  be  observed  carefully. 

In  children  one  grain  twice  a  day  should  be  the  maximum  dose  at  the  be- 
ginning ;  this  may  be  increased  to  three  or  five  grains  twice  daily  until  the 
amount  is  established  which  the  patient  needs  to  keep  in  a  normal  condition. 

A  word  of  caution  is  in  order  not  to  continue  the  remedy  if  muscular  pain 
and  cardiac  attacks  occur.  In  several  cases  I  have  been  compelled  to  discon- 
tinue the  use  of  the  gland  on  account  of  extreme  and  rapid  emaciation. 
Tonic  measures  should  be  employed  promptly  if  the  general  health  of  the  child 
has  been  impaired. 

ANGIONEUROTIC  (EDEMA. 

Angio-  Neurotic  (Edema  is  a  trophic  disorder,  first  described  by 
Quincke,  in  1882,  although  conditions  closely  resembling,  or  identical  with  it, 
have  been  known  ever  since  1827.  It  has  passed  under  the  designations  of 
"  acute  circumscribed  oedema,"  "  periodic  swelling,"  "  Australian  blight," 
"  non-inflammatory  oedema,"  etc. 

The  disease  is  characterized  by  the  appearance  of  circumscribed  swell- 
ings in  various  parts  of  the  body,  more  especially  in  the  face,  throat,  and  the 
extremities.  These  swellings  appear  without  any  direct  cause,  and  are  often 
associated  with  gastro-intestinal  disturbance,  which  is  thought  to  be  due  to 
a  condition  of  the  mucous  membrane  of  the  stomach  and  bowels  similar  to 
that  of  the  skin  or  larynx  when  these  parts  are  diseased.  The  disease  comes 
on  in  attacks,  which  are  precipitated  most  easily  by  exposure  to  cold,  and  by 
slight  or  severe  injuries.  The  attacks  are  most  apt  to  occur  between  the 
hours  of  1  and  5  a.m.  In  71  cases  the  parts  first  affected  were  :  The  face  in 
29  cases ;  the  extremities  in  22  cases ;  the  larynx  in  5  cases  ;  the  genitals 
— penis,  scrotum— in  3  cases  ;  the  trunk  in  6  cases  ;  the  stomach  in  3  cases ; 
the  gums,  neck,  ear,  each  in  1  case. 

The  oedema  may  be  fully  developed  within  one-half  to  two  hours.  The 
color  of  the  skin  is  either  whitish,  or  a  dull  roseate  hue  with  a  whitish  shad- 
ing near  the  centre  of  the  cedematous  patch.  There  is  no  pitting  on  pressure, 
and  none  of  the  signs  of  an  inflammatory  swelling.  The  swelling,  if  in  the 
face,  may  be  marked  enough  to  cause  disfigurement.  The  sudden  jumping 
of  the  swelling  from  one  part  of  the  body  to  another  is  quite  characteristic. 
Subjectively  there  is  a  sensation  of  scalding  or  burning,  or  an  itching  on  the 
part  affected  ;  and  after  the  oedema  subsides  a  "  heavy  wooden  "  feeling  is 
complained  of.     The  disease  is  not  a  serious  one  unless  it  affects  the  mucous 


202  THE  NERVOUS  DISEASES   OF  CHILDREN. 

membranes  of  the  larynx  and  pharynx ;  if  the  oedematous  swelling  in  the  larynx 
increases  rapidly,  death  may  take  place  from  asphyxia,  as  in  two  cases  re- 
ported by  Osier.  One  case  has  been  reported  that  proved  fatal  from  oedema 
of  the  glottis  (Krieger). 

The  gastro-intestinal  tract,  if  affected,  may  exhibit  severe  disturbance, 
such  as  pain  in  the  epigastrium,  vomiting,  colic,  retraction  of  the  abdomen, 
and  constipation  or  diarrhoea. 

OZdema  of  the  lungs  has  been  reported  as  a  symptom  of  this  disease  ;  but 
proof  of  this  is  wanting. 

Collins  has  analyzed  75  cases. 

According  to  this  author  the  average  age  at  the  onset  of  the  disease  is 
twenty-seven  ;  childhood  is  by  no  riieans  exempt.  Dinkelacker  has  reported 
the  case  of  a  child  that  had  its  first  attack  when  three  months  old  ;  similar 
cases  have  been  reported  by  Widonitz  and  Goltz.  The  disease  occurs  nearly 
twice  as  often  in  males  as  in  females.  It  may  come  on  after  any  exhausting 
condition ;  but  heredity,  as  well  illustrated  in  a  family  described  by  Osier,  is 
the  most  important  factor.  In  this  family  five  successive  generations  have 
been  affected.  Severe  emotional  excitement  and  hysterical  conditions  are 
the  forerunners  of  the  disease.  The  ingestion  of  certain  foods  was  followed 
by  this  special  form  of  oedema  in  cases  reported  by  Osier,  showing  an  etio- 
logical resemblance  at  least  between  angio-neurotic  oedema  and  urticaria. 

Matas  and  others  have  described  cases  of  this  disease  coming  on  after 
or  during  malarial  fever.  The  diagnosis  is  made  readily  enough  ;  it  may  be 
confounded  with  the  blue  oedema  of  hysteria,  as  described  by  Sydenham,  or  the 
white  oedematous  swellings  of  hysteria  described  by  Charcot ;  but  the  pres- 
ence or  absence  of  the  stigmata  of  hysteria  will  help  to  disprove  or  corrobo- 
rate the  suspicion  of  angio-neurotic  oedema. 

There  can  be  little  doubt  that  angio-neurotic  oedema  is  a  vasomotor 
neurosis ;  it  is  analogous  to  the  non-inflammatory  swellings  which  Weir  Mitch- 
ell described  after  injury  to  the  peripheral  nerves,  and  to  similar  swellings, 
which  appear  after  stretching  a  nerve.  I  have  seen  oedematous  swellings  in 
the  face,  after  section  of  the  trigeminal  nerve,  very  like  the  circumscribed 
oedema  under  discussion.  The  serous  exudation  in  all  probability  results 
from  a  retardation  of  the  blood-current,  and  this  in  turn  must  be  attributed 
to  a  local  paresis  of  the  vaso-constrictors,  or  a  reflex  stimulation  of  the  vaso- 
dilators. 

Treatment  of  this  disease  as  it  occurs  in  children  is  more  difficult  than  in 
the  adult,  in  whom  the  removal  of  toxic  causes,  such  as  alcohol  and  tobacco, 
is  all  that  is  needed.  In  children  the  hereditary  element  is  most  marked, 
and  all  we  can  hope  to  do  is  to  inhibit  the  attack  and  to  strengthen  the 
general  nervous  system.  To  check  the  attack  we  may  compress  the  affected 
part  by  an  elastic  bandage,  or  apply  dry  heat.  Small  doses  of  morphia  or 
codeia  may  be  given.  In  the  case  of  swelling  in  the  pharynx  or  larynx,  sur- 
gical interference  may  be  necessary.  To  improve  the  general  health  of  the 
child  it  will  be  best  to  administer  strychnia  in  small  doses  (grain  one-hun- 
dredth to  grain  one-sixtieth),  and  to  give  blood-tonics  such  as  iron  and  ar- 
senic. 


VASOMOTOR  AND   TROPHO-NEUROSES.  203 


RAYNAUD  S    DISEASE. 

Raynaud's  Disease,  or  symmetrical  gangrene,  is  a  very  rare  tropho- 
neurosis, which  occurs,  however,  quite  as  frequently  in  children  as  in  adults. 
Indeed,  says  Morgan,  "  if  there  be  any  period  when  it  is  especially  prone  to 
occur,  it  is  in  childhood."  Of  93  cases  which  he  analyzed  24  were  in  children 
under  ten  years  of  age,  and  5  of  Raynaud's  original  cases  were  children  be- 
tween three  and  nine  years  old.  Mendel  observed  a  case  in  a  child  fifteen 
months  old. 

The  symptoms  of  the  disease  are  practically  the  same  as  those  first  enu- 
merated by  Raynaud  in  1862.  A  localized  ischaemiaor  asphyxia  in  symmetri- 
cally situated  parts  is  the  most  constant  symptom  ;  this  asphyxia  may  lead  to 
gangrene,  but  does  not  invariably  do  so  ;  nor  is  the  disease  invariably  sym- 
metrical, and  for  that  reason  the  designation  of  symmetrical  gangrene  is  not 
entirely  appropriate.  The  order  of  development  of  the  symptoms  may  vary 
somewhat.  Before  the  local  syncope  is  fully  developed,  there  is  often  a 
general  numbness  and  some  pain  in  the  part  to  be  affected.  The  affected 
area  becomes  pale  and  waxy  in  appearance  ;  if  pricked,  little  or  no  blood  flows 
from  it.  This  condition  may  be  recovered  from,  and  several  such  attacks 
may  occur,  each  one  ending  in  recovery ;  finally,  however,  the  condition  per- 
sists, and  local  asphyxia  is  developed  ;  in  some  instances  the  condition  is 
reached  at  once  and  is  persistent.  The  affected  areas  become  deep  red,  then 
blue,  and  finally  black  in  color ;  the  parts  are  swollen,  and  the  local  temper- 
ature is  lowered.  Extravasation  of  blood  into  the  surrounding  tissues  may 
occur.  Recovery  at  this  stage  is  still  possible  ;  but  if  the  morbid  process  is 
continued  for  some  time  the  parts  grow  thinner,  the  fingers  become  atten- 
uated, and  finally  gangrene  results.  The  gangrene  comes  on  either  some 
months  after  the  first  symptoms  of  local  asphyxia,  or  it  is  developed  at  the 
earliest  period  of  the  disease.  The  tips  of  the  fingers  and  toes  may  be  de- 
stroyed by  this  process,  or  an  entire  hand  or  foot  or  some  other  considerable 
part  may  be  destroyed  by  gangrene.  The  parts  most  frequently  affected, 
are  the  fingers,  toes,  ears,  the  buttocks,  the  calves,  and  the  nose.  The  gan- 
grenous area  is  generally  separated  from  healthy  tissue  by  a  sharp  line  of 
demarcation ;  the  dead  part  may  either  become  mummified,  or  it  may  be 
cast  off  after  suppuration.  Moist  gangrene  also  occurs  at  times,  with  the  for- 
mation of  large  bullae  and  pus  centres.  If  a  gangrenous  area  heals,  it  behaves 
like  a  torpid  ulcer  (Elliot). 

Intermittent  haemoglobinuria  and  glycosuria  have  been  observed.  The 
pathology  of  the  disease  is  still  obscure.  Raynaud  and  his  successors  were 
of  the  opinion  that  the  local  syncope  is  produced  by  a  contraction  or  spasm 
of  the  blood-vessels  (both  arteries  and  veins).  The  disease  of  the  blood- 
vessels is  by  several  authors  attributed  to  syphilis  or  Bright 's  disease.  The 
symptoms  of  Raynaud's  disease  have  also  been  observed  in  association 
with  tabes,  syringomyelia,  myelitis,  and  neuritis. 

The  prognosis  is  generally  good  ;  if  death  occurs,  it  is  due  to  some  in- 
tercurrent disease.     Recoveries  are   frequent,  even  if  local  gangrene  has 


204  THE  NERVOUS  DISEASES   OF   CHILDREN. 

existed ;  but  if  large  portions  become  gangrenous,  the  general  health  of  the 
patient  may  be  seriously  impaired.  In  such  cases  tuberculosis  may  be  de- 
veloped and  hasten  death. 

Treatment  should  be  directed  toward  the  improvement  of  the  patient's 
general  condition,  and  freedom  from  all  emotional  excitement  should  be  in- 
sisted upon.  The  affected  parts  should  be  wrapped  in  cotton,  and  placed  in 
a  position  most  favorable  for  the  circulation.  Dry  heat  or  warm  baths  may 
be  employed  to  sustain  the  temperature  of  diseased  areas.  Nitro-glycerine 
and  nitrite  of  amyl  have  been  recommended,  but  they  have  not  been  exhib- 
ited successfully.  Electric  (galvanic)  baths,  and  the  application  of  the  gal- 
vanic current  through  the  cervical  spine  and  the  affected  area  have  also 
been  favored  by  some ;  but  very  little  good  has  resulted  from  such  treatment. 
The  greatest  benefit  will  be  derived  from  surgical  measures  after  gangrene 
has  set  in. 

FACIAL   HEMIATROPHY. 

Facial  Hemiatrophy  is  a  rare  form  of  disease.  Often  it  begins  in 
childhood,  and  has  important  relations  to  other  diseases  of  the  nervous  sys- 
tem. It  has  been  described  by  some  with  exophthalmic  goitre  and  with  mi- 
graine, but  its  relations  appear  to  me  to  be  very  much  closer  to  scleroderma, 
which  occasionally  precedes  it,  and  I  have  recently  seen  a  case  of  universal 
scleroderma  in  which  there  was  double  hemiatrophy  of  the  face. 

The  disease  is  characterized  by  a  distinct  diminution  in  the  size  and  bulk 
of  the  subcutaneous  tissues,  and  by  the  alteration  in  the  bony  structure  as 
well.  In  one  case,  on  the  left  side  the  distance  from  the  middle  of  the  chin  to 
the  angle  of  the  jaw  was  1 1  ctm. ;  on  the  right  side,  i2l/&  ctm. ;  from  the  upper 
margin  of  the  naso-labial  fold  to  the  middle  of  the  ear  was  10^  ctm.,  while 
on  the  right  side  this  distance  measured  1 1  %  ctm.  The  first  indications  of 
atrophy  are  found  in  the  skin.  A  single  spot,  generally  in  the  cheek,  be- 
comes thin  and  white  from  the  disappearance  of  pigment.  The  loss  of  fatty 
tissue  underneath  causes  a  depression  also  at  this  point.  The  atrophy  pro- 
ceeds from  this  and  gradually  involves  the  entire  half  of  the  face.  The  eye 
is  retracted,  there  is  distinct  wasting  of  one-half  of  the  nose,  and  even  the 
ear  of  one  side  may  be  smaller  than  that  of  the  other  side.  In  some  few  in- 
stances a  slight  glossiness  of  the  skin  has  been  observed,  but  in  the  majority 
of  cases  there  is  unusual  dryness,  the  condition  resembling  scleroderma. 
The  hair  also  is  apt  to  be  stiff  and  hard.  In  some  instances  the  teeth  in 
the  affected  half  are  small,  and  undergo  decay.  There  is  no  disturbance 
of  sensation,  and  no  interference  with  any  of  the  special  senses  in  the  ordi- 
nary run  of  cases.  The  temperature  sometimes  varies  a  little.  In  a  case 
which  I  reported  the  temperature  was  one  degree  higher  in  the  normal  ear 
than  in  the  ear  of  the  affected  side.  The  muscular  movements,  in  spite  of 
the  increasing  atrophy,  as  a  rule,  remain  normal.  Several  other  authors, 
and  myself,  have  reported  cases  in  which  there  were  distinct  tonic  and 
clonic  contractions  of  the  muscles  supplied  by  the  fifth  nerve  of  the  affected 
side.  Atrophy  of  one-half  of  the  tongue  also  occurs  in  some  cases,  but  it  is 
a  rare  complication. 


VASOMOTOR   AND   TROPIIO-NEUROSES.  205 

The  causes  of  the  disease  are  unknown,  except  that  several  cases  have 
been  developed  after  severe  injuries  to  the  face.  Thus  in  one  case  re- 
ported by  Skryme,  and  quoted  by  Gowers,  in  a  child  three  and  a  half  years 
of  age,  the  disease  was  developed  six  months  after  an  accident  in  which  "  the 
face  was  jammed  and  the  neck  twisted."  The  disease  has  also  been  devel- 
oped after  acute  infectious  diseases,  and  after  other  causes  which  give  rise  to 
neuritis.  There  can  be  no  doubt,  however,  that  in  many  cases  the  disposi- 
tion to  the  disease  is  congenital. 

Pathology. — There  was  much  dispute  regarding  the  origin  of  this  dis- 
ease, some  claiming  that  the  cervical  sympathetic  was  responsible,  others 
making  it  a  disease  of  the  trigeminal  nerve.  There  is  no  doubt  that  a  con- 
dition very  similar  to  hemiatrophy  will  follow  upon  lesions  of  the  cervical 
sympathetic ;  but  an  autopsy  performed  by  Mendel  on  a  case  which  had 
been  observed  by  many  other  men,  proved  beyond  a  doubt  that  in  that  case, 
at  least,  the  disease  was  due  to  a  proliferating  interstitial  neuritis  of  the  left 
fifth  nerve,  and  that  this  neuritis  was  most  marked  in  the  second  branch ;  in 
that  case  the  facial  nerve  was  entirely  normal,  but  the  left  musculo-spiral  had 
undergone  the  same  changes  as  the  left  trigeminal.  My  own  case,  in  which 
there  were  marked  clonic  and  tonic  contractions  of  the  masseters,  would  also 
tend  to  show  that  the  trigeminus  was  the  chief  seat  of  the  trouble,  and  that 
in  some  instances  the  motor,  as  well  as  the  trophic  fibres  in  the  trigeminus 
can  be  involved  in  the  same  morbid  process.  The  occurrence  of  a  neuritis 
of  other  nerves,  as  well  as  the  association  of  facial  hemiatrophy  with  sclero- 
derma, would  seem  to  prove  that  the  morbid  process  may  in  some  cases  ex- 
tend beyond  the  fifth  nerve. 

The  course  of  the  disease  is,  as  a  rule,  steadily  progressive,  though  after 
the  lapse  of  time  there  may  be  a  complete  standstill  of  all  the  symptoms. 
The  disease  does  not  call  for  any  active  treatment,  excepting  in  cases  like  my 
own,  in  which  the  spasmodic  contraction  of  the  masseters  was  extremely 
painful  and  had  to  be  relieved  by  opiates.  The  disfigurement  of  the  face  is 
the  most  serious  feature  of  the  disease.  In  the  case  of  one  patient  I  suc- 
ceeded in  rounding  out  the  cheek  again  by  inserting  a  light  rubber  plate, 
which  was  attached  by  a  dentist  to  the  patient's  upper  teeth.* 

*  In  view  of  the  excellent  results  obtained  by  thyroid  feeding  in  a  case  of  hemi- 
atrophy with  scleroderma,  this  treatment  deserves  a  fair  trial. 


PART  II. 

ORGANIC    DISEASES   OF  THE    NERVOUS 

SYSTEM. 


CHAPTER   XII. 

DISEASES   OF   THE   PERIPHERAL   NERVES. 

Traumatism,  toxic  infections,  exposure  to  wet  and  cold, 
rheumatism,  the  causes  which  lead  to  disease  of  the  pe- 
ripheral nerves  in  the  adult  are  not  frequent  in  children.  A 
description  will  be  given  of  those  forms  only  which  are 
commonly  met  with  in  the  earlier  years  of  life. 

In  diseases  of  the  peripheral  (mixed)  nerves,  whether 
there  be  an  inflammatory  or  degenerative  process,  the  symp- 
toms are,  as  a  rule,  motor  and  sensory  in  character.  The 
paralysis  is  limited  to  the  muscles  supplied  by  the  diseased 
nerve  and  by  branches  coming  off  below  the  site  of  injury  or 
disease.  The  sensory  symptoms  may  be  a  hyperesthesia  or 
an  anaesthesia,  according  to  the  degree  of  change.  Pares- 
thesias are  very  common,  and  if  the  nerve  is  inflamed  it  is 
painful  on  pressure.  It  is  a  striking  fact,  however,  that  the 
motor  fibres  suffer  much  more  readily  than  the  sensory  fila- 
ments;  possibly  because  the  former  are  farther  removed 
from  the  nutrient  cell,  and  probably  because  a  collateral  in- 
nervation between  sensory  fibres  is  more  easily  established. 
Sensory  fibres  are  also  regenerated  more  easily.  Further 
symptoms  are,  marked  atrophy,  loss  of  reflexes  in  the  dis- 
eased member,  and  changes  in  electrical  reaction.  The 
degree  to  which  the  last  are  developed  gives  an  indication 
of  the  amount  of  degeneration  in  the  nerve. 

Trophic  disturbances  are  very  common  in  connection 
with  peripheral  neuritis  and  degeneration  of  peripheral 
nerves ;  but  these  disturbances  do  not,  as  a  rule,  appear  ex- 
cept in  severe  cases.  Local  cyanosis,  oedema,  and  ulcers  are 
common ;  the  skin  becomes  smooth  and  glossy,  and  the 
growth  of  the  nails  may  be  interfered  with. 


2IO 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


In  children,  as  in  adults,  the  nerves  of  the  brachial  plexus 
are  most  frequently  affected. 

The  symptoms  referable  to  lesions  of  the  brachial  plexus 
vary  according  to  the  part  of  the  plexus  involved.  As  in  the 
adult,  we  can  distinguish  between  several  distinct  types ; 
the  upper-arm  type,  the  lower-arm  type,  and  the"  paralysis 
due  to  total  plexus  lesion. 


THE   OBSTETRICAL   PALSIES. 


As  the  name  implies,  these  palsies  are  due  to  manipula- 
tions during  labor.     If  the  shoulder  and  clavicle  are  pressed 


Jtnt.TJieracic 


XSupra-Scap. 


X  Circumflex. 
M 'Muse.  Cut. 


"Piral. 


Fig.  53. — The  Brachial  Plexus :  the  Branches  involved  in  the  Upper  Arm  Type  of 
Paralysis  are  indicated  by  Shading. 

backward  and  upward  injury  to  the  plexus  may  result  there- 
from. In  cases  of  breech  presentation  pressure  upon  the 
supra-clavicular  spaces  may  cause  a  partial  plexus  paralysis. 
I  have  seen  several  cases  in  which  this  paralysis  was  bilat- 
eral. The  disturbances  that  ensue  upon  these  obstetrical 
manipulations  may  be  the  result  of  a  simple  traction  upon 
or  of  laceration  (Pierce  Clark)  of  some  or  of  all  the  fibres 
of  the  brachial  plexus  (Erb's  type  most  frequent). 

The  affected  arm  hangs  down  limp.  Although  the  child 
moves  the  fingers  and  the  arm  of  the  normal  side,  there 
is  little  or  no  power  on  the  paralyzed  side.     In  the  first 


DISEASES   OF   THE   PERIPHERAL   NERVES.  211 

few  weeks  after  birth,  if  electrical  examination  is  attempted, 
it  will  be  found  that  the  muscles  of  the  affected  extremity 
do  not  respond  to  the  electric  currents.  At  later  periods, 
in  the  milder  cases,  the  galvanic  response  of  the  nerves  may 
be  a  little  altered,  but  the  muscles,  as  a  rule,  do  not  exhibit 
any  marked  change.  If  the  case  is  a  more  severe  one  the 
diseased  branches  of  the  brachial  plexus  will  fail  to  respond 
both  to  the  galvanic  and  faradic  current,  and  the  muscles 
supplied  by  these  branches  will  show  either  an  altered 
galvanic  response,  the  anodal  closure  contractions  being 
greater  than  the  cathodal  closure  contractions,  or  the  af- 
fected muscles  may  fail  to  respond  altogether  to  the  inten- 
sity of  current  which  can  be  employed  in  children.  The 
ordinary  tests  for  sensory  disturbances  cannot  easily  be  ap- 
plied in  very  young  children.  There  is  generally  some 
slight  diminution  in  all  forms  of  sensation,  but  we  can  only 
state  with  certainty  that  the  sensations  of  touch  and  of  pain 
are  impaired  in  all  but  the  mildest  cases.  In  considering 
the  imperfect  sensation  of  a  very  young  child  we  must  bear 
in  mind  that,  as  Soltman  and  Westphal  have  shown,  the 
peripheral  nerves  are  not  fully  developed  until  several 
weeks  after  birth.  Evidently  the  entire  central  and  periph- 
eral apparatus  conducting  sensation  is  developed  later  than 
that  transmitting  motion. 

The  appearance  of  the  affected  limb  changes  a  little  as 
time  goes  on.  In  some  of  the  milder  forms  of  obstetrical 
paralysis  a  complete  restoration  takes  place  within  a  few 
weeks,  or  a  few  months,  with  or  without  medical  interfer- 
ence. In  the  severer  forms  the  atrophy  is  apt  to  increase  ; 
the  development  of  the  limb  is  retarded  as  compared  with 
the  normal  side.  It  is  common  enough  to  have  contract- 
ures at  the  elbows  and  at  the  wrists  develop  in  the  course 
of  a  few  months,  or  after  a  year  or  more,  in  those  cases  in 
which,  either  from  lack  of  treatment  or  from  the  severity  of 
the  injury  at  birth,  the  initial  paralysis  remains  unaltered. 

Pathology. — It  is  naturally  a  difficult  matter  to  deter- 
mine the  exact  changes  which  take  place  in  any  part  of  the 
brachial  plexus  in  consequence  of  obstetrical  manipulations, 
for  the  opportunities  of  a  post-mortem  examination  are 
very  rare.     Roger   describes   a  case   in   which   the  facial 


212  THE  NERVOUS  DISEASES   OF   CHILDREN. 

nerve,  and  the  arm  of  the  same  side  were  paralyzed  imme- 
diately after  birth  from  pressure  of  the  forceps.  Large 
extravasations  of  blood  were  found  post  mortem  in  the 
vicinity  of  the  stylomastoid  foramen,  and  of  the  brachial 
plexus.  Oppenheim,  who  examined  one  of  Henoch's  cases, 
discovered  degenerative  changes  in  the  brachial  plexus. 
In  the  milder  forms  of  these  peripheral  lesions  we  must 
suppose  that  the  disturbance  of  function  was  due  to  slight 
mechanical  injuries,  to  a  stretching  or  tearing  of  some  of 
the  fibres,  possibly  to  slight  injury  of  the  nerve-sheath,  or 
a  mild  form  of  inflammatory  reaction  in  the  nerve-tissue. 
In  the  severer  cases  the  lesion  may  amount  to  an  actual 
tear,  to  a  severance  of  the  nerve-fibres,  or,  in  the  case  of 
subluxation  of  the  head  of  the  humerus,  to  actual  compres- 
sion, with  loss  of  function,  of  the  nerve-fibres.  The  lower 
arm  type  (Klumpke)  of  brachial  plexus  lesion  has  been  re- 
ported. Also  a  case  of  laceration  of  the  spinal  roots  (Phil- 
ippe and  Cestan). 

Diagnosis. — The  differential  diagnosis  is  to  be  made 
between  these  obstetrical  palsies  and  the  so-called  "  birth 
palsies "  due  to  cerebral  lesions.  A  knowledge  of  the 
symptoms  due  to  lesions  of  the  motor  tract  will  be  useful. 

Peripheral  Palsies  (Obstetri-  Cerebral  Birth  Palsies, 

cal     Palsies     of     Brachial 
Plexus). 

1.  Arm  only  affected.  I.  Hemiplegia  or  diplegia  common  ; 

brachial  monoplegia  rare. 

2.  Flaccid  paralysis  with  atrophy.  2.  Spastic  paralysis,  with  or  without 

atrophy,  with  tendency  to  rigid- 
ity. 

3.  Deep  reflexes   absent,  surely  not      3.  Deep  reflexes  increased. 

exaggerated. 

4.  Changes  in  electrical  reaction  from      4.  No  changes  in  electrical  reaction. 

simple  loss  of  faradic  response  to 
complete  reaction  of  degenera- 
tion. 

5.  No  convulsions.  5.  Convulsions  apt  to  occur  and  to 

be  repeated. 

6.  Deformity  and  arrested  growth  of      6.  Flexion    contraction     of    fingers, 

entire  extremity.  wrist,  and  elbow. 

7.  Sensation  may  be  impaired.  7.  Sensation  not  affected. 


DISEASES   OF   THE   PERIPHERAL   NERVES.  21 3 

There  is  no  other  condition  from  which  these  obstetrical 
palsies  need  to  be  differentiated  if  the  child  is  examined 
within  a  reasonable  time  after  birth ;  but  if,  as  is  so  often 
the  case,  the  child  is  brought  to  a  physician  when  it  has 
reached  the  age  of  six,  seven,  or  even  twelve  years  of  age, 
the  true  cause  of  the  palsy  may  be  difficult  to  fathom.  At 
my  clinics  I  have  often  seen  cases  for  which  nothing  had 
been  done,  and  the  cause  is  elicited  only  after  the  closest 
inquiry  from  the  mother.  In  older  children  it  is  a  question 
whether  the  palsy  dates  from  birth,  or  whether  some  acci- 
dent in  the  first  few  years  of  life  may  not  have  been  the 
cause.  The  pulling  of  the  arm  in  some  of  the  games  the 
children  play,  wrenching  and  twisting  it  as  they  do  with 
considerable  force,  may  result  in  palsies  very  much  like 
obstetrical  palsies.  Poliomyelitis  is  not  easily  to  be  con- 
founded with  these  obstetrical  palsies,  for  the  former  is 
rare  in  the  first  year  of  life,  and  relatively  rare  in  the  upper 
extremities ;  the  atrophy  is  generally  more  extreme,  sensa- 
tion is  not  involved,  and  complete  recovery  is  not  common 
in  the  spinal  paralysis  of  children. 

Prognosis. — If  properly  cared  for,  recovery  may  be  ex- 
pected in  all  but  the  most  severe  forms  of  obstetrical  pe- 
ripheral palsy.  According  to  the  severity  of  the  symptoms, 
the  length  of  time  that  it  will  take  before  recovery  sets  in 
can  also  be  foretold,  with  some  degree  of  certainty,  and 
for  this  purpose  the  electrical  behavior  of  the  nerves  and 
muscles  constitutes  a  fair  test  for  the  amount  of  damage  that 
has  been  done.  Even  though  the  paralysis  be  complete,  if 
the  faradic  response  of  the  branches  supplying  the  affected 
muscles  is  not  entirely  lost,  the  probability  is  that  complete 
recovery  will  set  in  within  a  period  of  two  to  three  months. 
If  the  faradic  response  is  lost,  but  the  galvanic  formula  is 
not  altered,  the  restoration  of  function  may  be  expected 
within  a  period  of  six  months ;  but  if  both  the  faradic  and 
the  galvanic  responses  are  entirely  gone  or  seriously  im- 
paired, it  may  be  a  year,  or  even  two  years,  before  the  arm 
can  be  properly  used.  In  the  severer  cases,  in  which  a 
great  deal  of  injury  has  been  done  to  the  brachial  plexus, 
it  will  not  be  well  to  promise  much  regarding  the  time  or 
the  possibility  of  complete  recovery.     Some  of  these  cases 


214  THE  NERVOUS  DISEASES   OF   CHILDREN. 

are  extremely  stubborn  ;  this  is  to  be  emphasized,  for  the 
majority  of  authors  seem  to  regard  these  conditions  too 
favorably.  Much  will  depend  upon  the  accuracy  of  treat- 
ment. One  point  which  I  wish  to  impress  upon  the  reader, 
however,  is  that  in  a  fair  proportion  of  all  these  cases  the 
physician  may  reassure  the  parents  regarding  the  future 
condition  of  the  paralyzed  extremity. 

Treatment. — If  the  child  is  seen  immediately  after 
birth,  and  the  diagnosis  has  been  properly  made,  the  best 
the  physician  can  do  is  to  let  the  limb  severely  alone  for  a 
period  of  at  least  two  weeks ;  at  the  end  of  that  time  the 
exact  damage  that  has  been  done  can  be  properly  estimated 
and  the  necessary  therapeutic  measures  can  be  employed. 
Wrapping  up  the  arm  carefully,  so  as  to  avoid  any  further 
injury  to  it,  and  allowing  it  to  be  bathed  as  the  rest  of  the 
body  is,  will  be  all  that  is  necessary.  After  a  period  of 
two  weeks  I  would  advise  the  use  of  light  friction  and 
massage,  so  as  to  keep  up  the  nutrition  of  the  muscles,  and 
I  am  also  in  favor  of  directing  an  intelligent  nurse  or  rela- 
tive to  move  the  parts  systematically  so  as  to  overcome 
any  tendency  to  contracture.  After  a  period  of  four  to 
six  weeks  electricity  may  be  employed,  simply  as  a  mode  of 
exercise.  If  the  parts  respond  to  a  mild  faradic  current 
such  may  be  employed,  but  if  the  faradic  current  fails  to 
elicit  a  response  it  is  sheer  waste  of  time  to  employ  this 
form  of  electricity,  and  it  will  be  better  to  use  a  mild  gal- 
vanic current,  using  that  pole  which  will  produce  contrac- 
tions. Sittings  of  from  five  to  ten  minutes  every  day,  or 
every  other  day,  are  quite  sufficient,  and  will  be  of  'some 
benefit  in  the  way  of  preventing  excessive  atrophy  and  of 
preventing  the  formation  of  contractures. 

As  soon  as  there  is  a  tendency  to  the  development  of 
contractures,  it  will  be  well  to  place  the  arm  in  a  splint 
in  such  a  way  as  to  oppose  the  contracture  by  simple  me- 
chanical force,  and  to  take  the  arm  out  of  the  splint  daily 
for  the  purpose  of  treating  it  by  proper  massage  and  elec- 
tricity. If  deformities  have  arisen  as  the  natural  result  of 
disease,  or  from  neglect,  the  child  should  be  placed  in  the 
hands  of  an  orthopedic  surgeon  and  an  attempt  should  be 
made  by  every  known  surgical  procedure  to  correct  the 


DISEASES   OF  THE  PERIPHERAL  NERVES.  21  5 

existing  trouble.  The  old  belief  that  a  child  will  outgrow 
such  deformities  is  absolutely  unfounded.  It  is  an  unfortu- 
nate fact,  but  true,  nevertheless,  that  children  outgrow  only 
the  very  mildest  disturbances  of  the  nervous  system,  which 
is  equivalent  to  saying  that  some  troubles  undergo  spon- 
taneous cure.  Clark  and  others  have  advised  suturing  of 
the  lacerated  strands  in  severe  cases ;  some  favorable  re- 
sults have  been  reported.  Great  caution  should  be  exer- 
cised in  the  selection  of  cases. 

Paralysis  of  the  lower  limbs  due  to  obstetrical  manipulations  is  reported 
every  now  and  then,  but  it  is  extremely  rare.  The  lumbar  and  sacral  plexuses 
are  so  well  protected  at  every  point  of  emergence  from  the  spine  and  in  their 
course  through  the  gluteal  and  pelvic  regions  that  they  are  not  easily  disturbed 
by  any  amount  of  manipulation  or  traction  upon  the  legs.  An  injury  of  these 
parts  is,  however,  conceivable.  If  present,  the  paralysis  will  resemble  the 
paralysis  of  the  adult  in  the  number  of  muscles  involved  and  in  the  character 
of  the  palsy.  It  is  interesting  in  this  connection  to  note  that  Ross,  many 
years  ago,  supposed  that  traction  of  the  legs  was  occasionally  responsible  for 
spastic  paraplegia  in  children.  He  does  not  refer  to  the  effect  upon  the 
nerve-roots,  but  supposes  that  such  traction  would  be  followed  by  derange- 
ment of  the  fibres  in  the  spinal  cord  itself.  This  view  has  not  been  accepted, 
and  it  has  been  shown  that  cases  of  spastic  paraplegia  due  to  traumatism 
during  birth  are  due,  in  the  vast  majority  of  cases,  to  cerebral  and  not  to 
spinal  injuries.     Some  exceptions  have  been  credibly  reported. 

Still  another  obstetrical  palsy  occurring  quite  exceptionally  is  facial  paral- 
ysis due  to  compression  of  the  peripheral  portion  of  the  nerve  by  the  blade  of 
the  forceps.  This  is  an  unusual  occurrence,  for  if  the  blades  of  the  forceps 
are  properly  applied  they  should  not  be  in  the  vicinity  of  the  facial  nerve ; 
but  accidents  will  happen  and  the  blades  occasionally  slip  even  if  applied  by 
skilful  obstetricians,  and  thus  cases  of  obstetrical  facial  nerve  palsy  are  not 
entirely  unknown.  This  may  be  the  result  of  actual  pressure,  and  in  other 
cases  an  extravasation  of  blood  into  the  region  of  the  parotid  is  likely  to  com- 
press the  nerve  sufficiently  to  cause  temporary  palsy.  Exostoses  within  the 
pelvis  or  hasmatomata  may  cause  facial  palsies  by  direct  pressure  (Vogel,  E . 
Kehrer,  Schultze).  Schiitze  and  A.  Stein  have  reported  facial  and  hypoglossal 
palsy,  due,  presumably,  to  the  rare  coincidence  of  an  external  haematoma  and 
a  small  hemorrhage  into  the  hypoglossal  nucleus  (during  normal  labor) . 

The  prognosis  in  these  cases  is,  on  the  whole,  favorable.  In  a  few  cases, 
however,  a  degeneration  of  the  nerve  occurs  in  consequence  of  the  severity  of 
the  compression.  Henoch  reports  two  such  cases.  Parrot  and  Troisier 
have  examined  such  nerves  post  mortem.  I  have  the  records  of  two  cases, 
one  in  a  girl  of  twelve,  the  other  in  a  woman  of  some  twenty-two  years 
of  age,  in  whom  facial  palsy  with  unequal  development  of  the  two  halves 
of  the  face  is  the  result  of  this  obstetrical  accident. 


2l6 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


The  treatment  of  these  cases  is  exactly  the  same  as  that  for  all  other  pe- 
ripheral palsies.  Light  massage  and  the  use  of  the  electric  current  are  the 
only  measures  that  can  be  safely  recommended. 

FACIAL   PALSY   (BELL'S   PARALYSIS). 

Disease  of  the  seventh  nerve  is  not  infrequent  in  children 
from  the  same  causes  that  result  in  facial  paralysis  in  the 
adult.  We  have  to  con- 
sider, first,  rheumatic  fa- 
cial palsy,  more  properly 
perhaps  refrigeration  pal- 
sy ;  second,  facial  palsy 
due  to  ear  disease ;  and 
third,  facial  palsy  as  an 
accompaniment  of  disease 
at  the  base  of  the  brain. 

Rheumatic  facial  pal- 
sy may  occur  in  children 
at  almost  any  age.  Al- 
though I  have  seen  it  in 
a  child  nine  months  old, 
it  is  rare  before  the  age 
of  three  years,  and  is  most 
frequent  between  the  ages 
of  six  and  fifteen  years. 
Sudden  draughts  of  cold 
air  are  by  far  the  most 
frequent  cause.  The  side 
of  the  face  exposed  to  the 
draught  is  generally  the 
one  that  is  paralyzed.  In 
a  few  instances  it  is  diffi- 
cult to  make  out  the  ex- 
act cause,  but  even  under 
such  circumstances  re- 
frigeration is  very  prob- 
able. I  am  not  aware  that 
facial  palsy  occurs  in  connection  with  the  various  diatheses 
in  children  as  it  does  in  the  adult,  in  whom  it  is  met  with  in 
association  with  diabetes  or  tuberculosis. 


Fig.  54. — Diagrammatic  figure  showing  the  Re- 
lations of  the  Seventh  and  Glosso-Pharyngeal 
Nerves  and  the  Course  of  the  Taste  Fibres. 
The  lines  1,  2,  3,  4  mark  off  different  segments 
of  the  nerve.  N.f.,  facial  nerve  ;  N.  a..  Acous- 
tic nerve  ;  G.  ph. ,  glossopharyngeal  nerve  ; 
G.  £■.,  geniculate  ganglion  ;  N.  p.  j.,  great  su- 
perficial petrosal  from  fifth  nerve  ;  N.  c.  c.p.  £, 
tympanic  plexus  ;  the  dotted  line  S.  p.  s.  indi- 
cates salivary  fibres  ;  the  other  dotted  line  in- 
dicates taste  fibres;  Ch.  /.,  chorda  tympani ; 
N.  st. ,  stapedius  nerve  ;  F.  st. ,  stylo-mastoid 
foramen.     (Modified  from  Erb  by  Dana.) 


DISEASES   OF   THE   PERIPHERAL   NERVES.  21 J 

The  symptoms  of  facial  palsy  will  be  readily  under- 
stood by  an  examination  of  Fig.  54.  The  distal  portion  (1) 
is  the  one  which  is  generally  affected  in  these  rheumatic 
cases.  All  the  branches  of  the  facial  nerve  are  involved, 
but  there  is  no  interference  with  taste,  with  hearing,  with 
movement  of  the  palate,  or  with  the  secretion  of  saliva. 
The  paralysis  involves  the  muscles  of  the  eyes,  the  nose, 
the  cheeks,  and  the  lips.  On  inspection  of  the  child's  face  it 
is  easy  to  make  out  which  side  is  affected,  as  the  eyelids  of 
the  diseased  side  are  wide  open  (lagophthalmos).  If  the 
child  is  asked  to  close  the  eyes  it  does  so  with  ease  on  the 
sound  side,  but  the  diseased  side  fails  to  respond.*  In 
this  effort  there  is  over-action  of  the  frontal  muscles  and 
of  the  corrugator  supercilii  on  the  sound  side  ;  the  diseased 
side  showing  either  diminished  action  or  a  total  paralysis. 
The  child  cannot  pull  up  its  nose  ;  in  blowing  out  the  cheeks 
the  diseased  side  is  flabbier  than  the  sound  side,  and  if  the 
attempt  is  made  to  whistle  or  to  pout  the  lips,  as  in  kissing, 
the  insufficient  action  of  the  muscles  on  the  diseased  side 
becomes  very  evident.  If  asked  to  show  the  teeth,  the 
muscles  of  the  sound  side  overact,  and  the  mouth  is  drawn 
toward  the  healthy  side.  By  this  movement  the  paralysis 
is  often  revealed,  even  if  the  parts  appear  perfectly  normal 
while  at  rest.  The  tongue  when  protruded  deviates  toward 
the  sound  side ;  this  deviation  was  formerly  considered  to 
be  apparent,  not  real ;  but  Hitzig  has  shown  that  there  is 
an  actual  deviation  toward  the  sound  side,  and  supposes 
that  this  is  due  to  the  fact  that  the  tongue  wishes  to  avoid 
contact  with  the  corner  of  the  mouth  on  the  paralyzed  side. 
Contractures  of  the  muscles  of  the  paralyzed  side  occur  in 
the  later  stages  of  a  facial  palsy.  The  lower  branches  re- 
cover, as  a  rule,  more  slowly  than  the  upper,  and  in  several 
cases  I  have  observed  that  the  lower  and  middle  muscles 
could  be  contracted  only  if  the  attempt  was  made  to  close 
the  eyes  (Fig.  55). 

Sensory  disturbances  are  entirely  absent,  as  the  facial 
nerve  is  a  purely  motor  nerve,  the  sensory  fibres  of  the  face 

*  A  few  cases  of  double  facial  palsy  have  been  reported  (Mott,  Huebschman) ;  a  suc- 
cessive involvement  of  the  two  sides  is  more  frequent.  The  author  has  observed  it  in 
cases  associated  with  diabetes  in  the  adult. 


218 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


coming  from  the  fifth  nerve.  I  have  seen  a  few  cases  of 
severe  neuralgic  pains  complicating  facial  palsy  in  children, 
but  to  account  for  it  there  has  always  been  some  additional 
cause,  as  in  a  boy  of  fourteen,  whom  I  recently  examined, 
in  whom  decayed  upper  teeth  offered  the  explanation  for 
the  neuralgic  supraorbital  pain. 

Whatever  part  of  the  nerve  is  involved,  provided  it  be 
between  the  nucleus  of  the  facial  nerve  in  the  pons  and 
the  periphery,  electrical  changes  will  be  found.     In  all  but 


Fig.  55 — Boy  with  Facial  Palsy,  in  Stage  of  Recovery.  Slight  contracture  of  para- 
lyzed side  (B)  ;  in  figure  A  mouth  is  pulled  to  the  paralyzed  side  when  boy  attempts 
forcibly  to  close  left  eye  ;  also  deepening  of  naso-labial  fold. 

the  very  mildest  cases  the  faradic  response  of  the  nerve 
as  well  as  of  the  muscles  is  lost.  My  own  experience  has 
proved,  however,  that  there  is  an  exception  to  this  rule,  and 
that  is,  that  if  the  patient  be  examined  within  the  first  forty- 
eight  or  seventy-two  hours  after  the  onset  of  the  palsy,  the 
faradic  response  may  still  be  present ;  it  rapidly  diminishes, 
however,  and  after  a  period  of  three  days,  in  the  vast  ma- 
jority of  cases,  it  is  entirely  lost.  The  galvanic  response 
of  the  nerve  may  be  increased  during  the  first  few  days, 
but  it  is  soon  diminished  or  lost.  The  galvanic  irritability 
of  the  muscles  supplied  by  the  facial  nerve  shows  many  in- 
teresting changes.    In  the  earlier  stages  of  the  disease,  and 


DISEASES   OF   THE  PERIPHERAL   NERVES.  2 1 9 

in  all  but  the  severest  cases,  there  is  an  increased  galvanic- 
excitability  for  a  period  of  about  two  weeks.  The  same 
currents  which  produce  very  strong  contractions  on  the 
diseased  side  give  weaker  contractions  on  the  sound  side. 
This  can  be  easily  demonstrated  in  the  case  of  the  orbicu- 
laris oris.  If  a  very  weak  current  be  applied  to  the  sound 
half  of  the  muscle,  contractions  may  be  seen  in  the  diseased 
half  and  not  in  the  normal  half,  the  increased  excitability 
in  the  latter  being  so  great  that  the  small  amount  of  current 
diffusing  to  the  paralyzed  side  is  sufficient  to  produce  a 
contraction,  while  the  full  current  is  not  sufficient  to  make 
the  healthy  half  contract.  During  this  period  the  cathodal 
closure  contraction  of  the  diseased  muscle  is  generally 
greater  than  its  anodal  closure  contraction.  After  a  little 
while  the  increased  excitability  diminishes  and  soon  falls 
below  that  of  normal  muscular  tissue.  The  polar  con- 
tractions also  indicate  a  change,  the  cathodal  closure  con- 
traction first  being  equal  to  the  anodal  closure  contraction ; 
later  on  in  the  severer  cases  the  order  is  entirely  reversed. 

As  the  disease  approaches  the  period  of  recovery  the 
galvanic  formula  approaches  more  nearly  to  the  normal, 
the  cathodal  closure  contractions  being  equal  to  the  anodal 
closure  contractions.  Finally,  the  cathodal  closure  con- 
traction becomes  greater  than  the  anodal  contraction,  and 
the  contractions  that  have  been  sluggish  once  more  become 
prompt  and  short.  The  faradic  response  is,  as  a  rule,  rather 
longer  than  this  in  returning,  and  it  is  not  uncommon  to 
see  the  full  power  of  all  the  muscles  return  before  the  nor- 
mal electrical  reactions  have  been  established.  In  the  case 
of  a  boy  (Fig.  55)  the  faradic  response  of  the  muscles  and 
a  normal  galvanic  formula  were  not  restored  until  several 
months  after  the  contractures  had  set  in,  and  after  most  of 
the  muscles  could  be  innervated  tolerably  well. 

In  facial  palsy  due  to  ear  disease,  the  symptoms  can 
be  easily  accounted  for  by  the  position  of  the  nerve  in  the 
Fallopian  canal.  The  nerve  may  be  involved  during 
an  attack  of  otitis  media,  and  as  this  ear  affection  occurs 
often  enough  in  children  in  association  with  several 
acute  infectious  diseases,  notably  scarlatina,  this  form  of 
facial   palsy   is  quite  a  frequent  occurrence.     It  does  not 


220  THE  NERVOUS  DISEASES   OF   CHILDREN. 

occur  as  readily  in  the  earlier  stages  of  an  otitis  as  in  the 
later  suppurative  stages,  when,  in  consequence  of  caries  of 
the  bone,  the  facial  nerve  is  directly  involved.  According 
to  the  severity  of  the  disease  the  paralysis  will  be  more  or 
less  complete,  and  as  far  as  the  facial  nerve  is  concerned 
the  symptoms  will  resemble  those  of  the  rheumatic  form, 
but  naturally  the  association  of  facial  palsy  with  defective 
hearing  or  the  persistence  of  a  purulent  discharge  from  the 
ear  will  point  to  the  actual  cause  of  the  paralysis. 

I  have  seen  facial  palsy  occur  also  after  mastoid  opera- 
tions for  ear  disease.  In  these  cases  an  accident  during:  the 
operation  is  often  sufficient  to  break  through  the  fine  bony 
plate  separating  the  facial  nerve  from  the  ear  structures, 
and  a  more  or  less  severe  facial  palsy  is  the  result.  This 
occurred  in  a  little  patient  whose  case  I  reported  fifteen 
years  ago,  in  whom  the  mastoid  operation  was  done  for  the 
relief  of  epileptic  attacks  which  were  caused  by  the  reten- 
tion of  pus.  On  his  recovery  from  the  operation  a  distinct 
facial  palsy  on  the  side  of  the  operation  was  noticed,  and  ex, 
isted  for  a  period  of  very  nearly  three  months,  after  which 
complete  recovery  took  place. 

As  in  the  adult  so  in  the  child,  paralysis  of  the  face  may 
accompany  lesions  at  the  base  of  the  brain  and  in  the  pons. 
If  the  lesion  is  at  the  base,  other  cranial  nerves  are  involved 
as  well  as  the  facial,  and  the  diagnosis  can  therefore  be 
readily  made.  It  is  common  enough  to  find  the  facial 
nerve  partially  or  totally  paralyzed  in  cases  of  basilar  men- 
ingitis, whether  tubercular  or  non-tubercular.  In  cases  of 
injuries  to  the  skull,  or  of  tumors  occupying  the  middle  or 
posterior  fossa,  facial  palsy  is  one  of  a  large  series  of  symp- 
toms and  has  but  little  diagnostic  value  excepting  in  its  as- 
sociation with  affections  of  other  cranial  nerves.  It  may  be 
stated  in  this  connection  that  in  cases  of  cerebellar  tumor, 
which  are  by  no  means  rare  in  children,  both  the  acoustic 
and  the  facial  nerves,  which  lie  in  very  close  juxtaposition 
to  each  other  and  are  easily  pressed  upon  by  a  cerebellar 
growth,  are  often  paralyzed  and  give  rise  to  symptoms 
which  are  almost  pathognomonic  of  cerebellar  disease.  If 
the  lesion  is  in  the  pons,  and  particularly  if  it  be  below 
the  decussation  of  the  facial  nerves,  paralysis  of  the  face  is 


DISEASES   OE   THE   PERIPHERAL   JVERl  I  221 

associated  with  alternate  hemiplegia.  In  these  cases  every 
branch  of  the  facial  nerve  is  apt  to  be  involved.  II  the 
lesion  be  a  neoplasm  other  symptoms  pointing  to  compres- 
sion of  the  cranial  nerves  and  indicating  the  presence  of 
tumor  within  the  cranial  cavity  will  naturally  be  associated 
with  those  mentioned  before. 

The  diagnosis  of  facial  palsy  is  easy  to  make,  and  the  only  difficulty  that 
arises  at  times  is  to  determine  whether  the  palsy  is  indicative  of  a  central  or 
a  peripheral  lesion  ;  but  this  difficulty  can  be  readily  met  by  keeping  in  mind 
the  fact  that  if  the  lesion  is  in  the  brain  anywhere  above  the  pons  the  upper 
branches  of  the  facial  escape  altogether  and  the  electrical  responses  remain 
entirely  normal ;  furthermore  in  such  a  case  the  facial  palsy  is  associated  with 
hemiplegia  on  the  same  side  of  the  body  as  is  the  paralysis  of  the  face.  It  is 
far  more  difficult  to  determine  the  exact  part  of  the  nerve  that  is  diseased. 
As  the  intensity  of  the  affection  depends  somewhat  upon  the  extent  of  the 
nerve  tract  that  is  diseased,  this  inquiry  into  the  accurate  localization  of  the 
disease  has  a  practical  as  well  as  a  scientific  interest.  Taking  the  diagram 
on  page  216  we  can  study  the  variation  of  symptoms  according  to  the  part 
that  is  affected.  If  the  disease  is  limited  to  that  part  outside  of  the  Fallo- 
pian canal,  there  is  complete  paralysis  of  every  branch  of  the  facial  nerve 
with  the  exception  of  the  posterior  auricular.  Reflexes  are  wanting,  but  there 
are  no  disturbances  of  taste  or  hearing,  no  paralysis  of  the  palate,  and  no  dis- 
turbances of  salivary  secretion.  If  the  disease  happens  to  affect  the  part  of 
the  facial  nerve  as  far  as,  but  not  including,  the  chorda  tympani  (Fig.  54,  line 
2),  the  symptoms  are  paralysis  of  all  the  branches  of  the  facial,  including 
the  posterior  auricular. 

The  same  symptoms  as  the  above,  with  the  addition  of  a  disturbance  of 
taste  in  the  anterior  portion  of  the  tongue  and  a  diminution  in  salivary  secre- 
tion points  to  an  involvement  of  the  facial  nerve  from  the  point  of  junction 
of  the  chorda  tympani  to  the  junction  of  the  stapedius  with  the  main  trunk 
(from  2  to  3). 

If  that  part  of  the  nerve  lying  between  the  stapedius  and  the  ganglion 
geniculi  is  diseased,  the  symptoms,  in  addition  to  total  paralysis,  are  changes 
in  taste,  diminution  in  salivary  secretion,  and  abnormal  acuity  of  hearing,  but 
there  is  no  paralysis  of  the  soft  palate.  The  above  symptoms,  with  the  addi- 
tion of  paralysis  of  the  soft  palate,  point  to  a  lesion  in  the  region  of  the  gen- 
iculate ganglion  (division  4  to  5)  ;  but  on  this  point  there  is  much  doubt. 
See  table,  p.  13. 

Paralysis  of  all  the  peripheral  branches,  as  well  as  paralysis  of  the  soft 
palate,  abnormal  acuity  of  hearing,  decreased  salivary  secretion,  but  no  involve- 
ment of  taste,  help  to  locate  the  lesion  above  the  geniculate  ganglion,  and  be- 
tween it  and  the  exit  of  the  facial  nerve  from  the  brain  (division  5  to  6).  If  this 
part  of  the  facial  tract  is  diseased  other  cranial  nerves  are  frequently  affected. 
In  such  cases  we  are  apt  to  have  paralysis  of  the  abducens,  loss  of  hearing  on 
the  same  side  as  the  facial  palsy,  tinnitus  aurium,  and  the  reaction  of  degen- 


222  THE  NERVOUS  DISEASES   OF   CHILDREN. 

eration  of  the  acoustic  nerve.  If  we  have  total  palsy  of  every  branch,  palsy 
of  the  soft  palate  without  disturbance  of  taste  and  with  simple  diminution  of 
electrical  excitability,  without  any  other  marked  symptoms,  we  may  locate 
the  lesion  in  the  facial  nucleus ;  but  this  form  of  palsy  is  recognized  more 
definitely  if  other  cranial  nerves  are  simultaneously  affected,  such  as  the 
hypoglossal,  the  spinal  accessory,  the  vagus,  the  trigeminal  nerve,  etc. 

The  association  of  the  symptoms  just  mentioned  with  paralysis  of  the  arm 
and  leg  of  the  opposite  side,  puts  the  lesion  in  the  pons.  If  there  is  but  par- 
tial paralysis  of  the  face,  if  the  electrical  excitability  is  not  affected,  if  the  re- 
flexes are  normal,  and  if  the  extremities  of  the  same  side  are  affected,  the 
lesion  is  in  the  crus  or  in  the  hemispheres,  say  in  the  internal  capsule.  If  the 
facial  nerve  and  the  oculo-motor  nerve  of  opposite  sides  happen  to  be  affected 
the  lesion  is  positively  in  the  crus  on  the  same  side  as  the  ocular  palsy. 

The  course  and  prognosis  of  all  forms  of  facial  palsies 
will  depend  entirely  upon  the  nature  of  the  morbid  proc- 
ess. If  the  disease  is  due  to  caries  of  the  petrous  por- 
tion of  the  temporal  bone,  to  malignant  tumor,  to  fracture, 
to  tubercular  meningitis,  or  to  any  form  of  meningitis,  the 
prognosis  is  extremely  grave.  Even  those  cases  depending 
upon  specific  disease  at  the  base  are  not  as  amenable  to 
treatment  as  one  might  expect.  Those  due  to  parotitis  or 
to  internal  otitis  recover  if  the  disease  itself  is  not  of  the 
destructive  type.  Cases  due  to  simple  section  of  the 
nerve,  as  in  cases  of  operation,  yield  a  tolerably  favorable 
result. 

The  prognosis  of  the  rheumatic  forms  of  facial  palsy 
varies  according  to  the  intensity  of  the  disease,  but  it  does 
not  depend  apparently  upon  the  amount  of  nerve-tissue 
that  is  affected,  for,  on  the  whole,  cases  in  which  the  nerve 
within  the  canal  is  involved  take  about  as  favorable  a 
course  as  do  those  in  which  the  pes  anserinus  is  the  only 
part  affected.  Erb  distinguishes  three  forms — a  mild  form, 
a  moderate  form,  and  a  severe  form  of  facial  paralysis.  His 
division  depends  entirely  upon  the  electrical  conditions 
present  at  the  end  of  the  first  week.  I  cannot  agree  alto- 
gether with  his  statement  that  if  the  electrical  excitability 
is  entirely  normal  at  the  end  of  the  first  week,  as  in  light 
forms,  recovery  will  set  in  in  two  or  three  weeks.  If  at 
this  same  period  the  faradic  and  galvanic  excitability  are 
slightly  diminished  recovery  may  be  expected  in  from  four 
to  six  weeks,  and  if  at  the  end  of  the  first  week  the  elec- 


DISEASES    OF   THE   PERIPHERAL   NERVES.  223 

trical  excitability  of  the  nerve  is  very  much  diminished  or 
totally  lost,  the  disease  will  run  a  course  of  many  months. 
I  have  seen  cases  get  well  practically  within  four  weeks,  in 
which  the  faradic  and  galvanic  excitability  of  the  nerve- 
was  diminished  very  much  from  the  very  start,  and  I 
should  be  inclined  to  formulate  the  following  statements  : 

I.  If  at  the  end  of  the  first  week,  or,  still  better,  at  the 
end  of  the  second  week,  the  nerve  responds  at  all  to  the 
faradic  or  galvanic  current,  a  prompt  recovery  in  about 
four  weeks  may  be  expected. 

II.  If  at  about  the  same  time  the  nerve  fails  to  respond, 
but  the  muscles  show  a  diminished  or  altered  galvanic  re- 
sponse, the  disease  is  likely  to  run  a  course  anywhere  be- 
tween one  and  three  months. 

III.  If  the  muscles  respond  but  feebly  to  strong  cur- 
rents, if  the  galvanic  formula  is  altered,  and  if  the  contrac- 
tions are  extremely  slow,  the  disease  may  run  a  course  any- 
where between  six  months  and  a  year,  or  even  longer.  If 
after  a  period  of  two  months  no  electrical  reaction  can  be 
observed,  the  degeneration  is  very  complete,  and  a  paral- 
ysis lasting  at  least  a  year,  if  not  longer,  may  safely  be  pre- 
dicted. 

Treatment. — We  need  say  little  of  the  treatment  in  cases 
in  which  the  facial  palsy  is  only  one  of  many  symptoms 
pointing  to  gross  cerebral  disease.  Under  such  circum- 
stances treatment  directed  to  the  relief,  or  possibly  the  cure, 
of  these  conditions  will  have  to  be  employed.  In  the  cases 
of  facial  palsy  due  to  ear  disease,  proper  surgical  treatment 
should  be  recommended  at  a  very  early  day.  Much  doubt 
has  been  expressed  as  to  whether  any  treatment  can  cur- 
tail or  cure  the  rheumatic  palsies.  The  milder  forms  will 
undoubtedly  get  well  even  if  no  treatment  is  attempted. 
In  the  more  chronic  cases  the  use  of  the  electrical  current, 
particularly  of  the  galvanic,  can  be  safely  recommended, 
for  I  have  the  distinct  impression  that  cases  so  treated,  and 
particularly  those  that  have  been  properly  treated  from  the 
very  start,  run  a  somewhat  shorter  course  than  those  in 
which  no  electrical  treatment  has  been  attempted. 

In  the  year  1893  Remak  gave  statistical  evidence  in  favor 
of  this  view,  and  put  a  quietus  for  the  present  upon  those 


224  THE  NERVOUS  DISEASES   OF  CHILDREN. 

who  think  that  there  is  nothing  in  electro-therapeutics  save 
the  effect  of  suggestion. 

The  electrical  current,  even  if  it  have  but  slight  influ- 
ence in  restoring  the  power  of  conduction  in  the  diseased 
nerve,  is  surely  of  value,  as  it  is  in  paralysis  of  the  extremities, 
in  exercising  parts  that  cannot  be  moved  by  the  will,  and  for 
this  reason  if  for  no  other  it  deserves  to  be  employed. 

Counter-irritation  has  been  repeatedly  tried.  This  may 
be  accomplished  by  blistering  either  with  emplastrum  can- 
tharidum  just  in  front  of  the  ear  or  over  the  mastoid,  or  by 
the  use  of  the  actual  cautery.  I  am  free  to  confess,  however, 
that  the  value  of  counter-irritation  seems  questionable  in  all 
cases,  and  particularly  in  those  in  which  counter-irritation  is 
not  indicated  to  allay  pain  that  is  so  frequently  associated 
with  neuritis.  The  application  of  leeches  is  to  be  thoroughly 
condemned,  except  possibly  in  those  cases  in  which  the  fa- 
cial palsy  is  associated  with  ear  disease.  This  practically 
limits  us  to  the  use  of  electricity  as  the  only  therapeutic 
measure  which  promises  some  relief  in  the  severer  cases. 
In  these  cases  the  faradic  current,  as  recommended  by 
Erb  and  Duchenne,  may  be  employed  for  the  first  week 
or  two,  but  after  that  a  stabile  galvanic  current  applied 
over  the  mastoid  and  along  the  peripheral  divisions  of 
the  facial  nerve  is  more  in  order.  As  soon  as  the  ex- 
citability of  the  nerves  and  muscle  is  increased,  or  shows 
an  approach  to  the  normal,  regular  electrizations  of  the 
paralyzed  muscles,  in  sittings  of  ten  minutes  or  more,  two 
or  three  times  a  week,  should  be  practised.  I  wish  to  cau- 
tion the  physician,  however,  against  the  use  of  strong  cur- 
rents or  against  the  use  of  the  electrical  current  without 
the  safeguard  of  a  galvanometer.  A  current  varying  be- 
tween two  and  four  milliamperes  is  quite  sufficient  for  all 
purposes.  I  have  also  directed  my  patients,  as  soon  as  the 
least  power  returns,  to  practise  contraction  of  the  muscles 
(closing  of  the  eyes,  pouting  of  the  lips,  etc.),  while  look- 
ing into  a  mirror. 

As  for  medicinal  measures,  they  may  be  discarded  alto- 
gether. In  many  cases  I  have  tried  strychnia  both  per  os 
and  in  the  form  of  hypodermic  injections,  and  I  am  firmly 
convinced  that  the  effect  of  such  medication  is  absolutely 


DISEASES   OF  THE  PERIPHERAL  NERVES.  22  5 

nil,  nor  can  we  expect  much  more  from  the  use  of  irritat- 
ing ointments  or  douches  to  the  face.  If  drugs  must  be 
administered  one  may  exhibit  iron,  arsenic,  and  cod-liver 
oil.  In  inveterate  cases,  union  of  the  facial  with  the  spinal 
accessory  or  the  hypoglossal  nerve  may  be  attempted. 

Other  Peripheral  Palsies. — Almost  any  peripheral  palsy  which  is 
known  to  occur  in  the  adult  may  at  times  occur  in  the  child.  Of  these  I  will 
make  special  mention  only  of  a  peripheral  palsy,  generally  of  traumatic  ori- 
gin, affecting  the  peroneal  nerve.  Injury  to  this  nerve  occurs  readily  enough 
if  a  child  happens  to  be  violently  struck  upon  the  leg,  or  if  the  leg  happens 
to  be  squeezed  against  a  post,  a  chair,  or,  as  in  one  case  I  have  seen,  against 
the  edge  of  the  bed.  I  mention  this  special  palsy  because  it  may  lead  to 
slight  difficulties  in  diagnosis,  inasmuch  as  confusion  may  arise  with  acute 
anterior  poliomyelitis  which  affects  the  muscles  supplied  by  the  peroneal 
more  often  than  those  supplied  by  any  other  nerve. 

The  onset  of  the  paralysis  without  fever,  the  presence  of  considerable 
pain,  the  marked  and  rapid  tendency  to  recovery,  and  the  history  of  violent 
traumatism  will  help  to  distinguish  this  peripheral  palsy  from  the  essential 
paralysis  of  children.  The  course,  prognosis,  and  treatment  of  such  a  pero- 
neal neuritis  will  be  exactly  the  same  as  in  the  case  of  other  nerve  palsies. 

Spasm  of  various  peripheral  nerves  is  common  in  young  persons.  The 
facial  and  the  spinal  accessory  nerves  are  frequently  affected  ;  the  trigeminal 
in  rare  instances  only.  Spasm  of  the  facial  nerve  (tic  convulsif)  is  much 
less  frequent  than  in  adults ;  a  spasm  involving  an  entire  facial  nerve  of  a 
child  is  very  rare  indeed  ;  but  partial  spasms  are  observed  in  the  form  of  nic- 
titation (clonic  spasm)  or  of  blepharospasm  (tonic  spasm  of  the  obicularis 
palpebrarum).  These  movements  are  generally  due  to  the  influence  of  cold, 
to  irritation  of  the  trigeminus,  but  are  also  frequently  of  psychic  origin. 
In  the  cases  of  clonic  spasm  of  the  entire  nerve,  all  sorts  of  extravagant 
grimaces  are  indulged  in.  These  spasms  must  not  be  confounded  with  habit 
chorea. 

Spasm  in  the  distribution  of  the  spinal  accessory  nerve  involves  either  the 
sternocleido-mastoid  or  the  trapezius  (spasmodic  wryneck). 

The  spasm  may  be  reflex  in  origin,  or  due  to  irritation  of  the  nerve  in  its 
course  from  the  medulla  and  spinal  cord  to  the  periphery.  The  position  and 
movements  of  the  head  will  vary  according  to  the  muscles  involved.  These 
spasms  may  be  unilateral  or  bilateral. 

If  we  remember  that  spasm,  whether  clonic  or  tonic,  denotes  an  excess  of 
normal  function,  the  symptoms  can  be  easily  understood  by  reference  to  the 
tables  on  pages  15  and  16. 

Ordinary  wryneck,  so  frequent  in  children,  is  due  to  a  rheumatic  myo- 
sitis. Symptomatic  wryneck  occurs  in  connection  with  cervical  adenitis, 
abscesses  and  caries  of  the  cervical  spine.  Congenital  wryneck  is  due  to 
some  pre-natal  disturbance  ;  it  may  be  of  intra-uterine  origin,  and  denote  im- 
perfect development,  in  which  case  it  is  generally  associated  with  atrophy 


226  THE  NERVOUS  DISEASES   OF   CHILDREN: 

of  one-half  of  the  face  ;  at  times  it  is  due  to  obstetrical  injuries  in  cases  of 
breech  and  foot  presentations. 

The  treatment  of  spasms  of  the  peripheral  nerves  consists  in  the  use  of  sed- 
atives, such  as  opium,  conium,  codein,  chloral,  and  bromides  ;  in  some  cases 
the  valerianate  of  zinc,  or  the  ammoniated  tincture  of  valerian,  might  be  given. 
The  galvanic  current  may  be  applied  in  the  milder  cases.  The  attempt  may 
be  made  to  check  the  spasms  by  hypodermic  injections  of  hydrobromate  of 
hyoscine,  one  two-hundredth  to  one  one-hundredth  grain,  or  by  the  use  of 
atropia  (one-fiftieth  to  one-tenth  grain,  as  recommended  by  Leszynsky). 

Tenotomies  are  in  order  in  the  congenital  and  chronic  cases.  Keen  has 
suggested  resection  of  the  posterior  branches  of  the  upper  three  or  four 
cervical  roots ;  but  the  advisability  of  this  procedure  is  still  in  doubt. 

Spasm  of  the  hypoglossal  nerve  I  have  not  seen  in  children.  The  com- 
plex co-ordinated  spasms  have  been  considered  in  Chapter  VI. 


CHAPTER   XIII. 

MULTIPLE    NEURITIS. 

Neurology  has  few  more  signal  achievements  to  show 
than  the  advances  that  have  been  made  in  the  study  of 
multiple  neuritis  within  the  past  decade.  Many  conditions 
which  were  formerly  supposed  to  be  due  to  an  affection 
of  the  spinal  cord,  and  which  were  often  confounded  with 
poliomyelitis,  and  with  various  forms  of  acute  and  sub- 
acute myelitis,  are  now  positively  known  to  be  due  to  poly- 
neuritis. The  term  multiple  neuritis  or  polyneuritis  is 
used  to  embrace  all  those  diseases  in  which  the  symptoms 
are  due  to  disease  of  several  or  many  of  the  nerves  of  the 
body.  There  is  not  only  a  tendency  to  multiplicity  but 
also  a  tendency  to  symmetrical  development. 

Many  of  the  older  writers  recognized  the  possibility  of  multiple  nerve  af- 
fections, and  a  multiple  neuritis,  as  such,  was  recognized  by  Dusmenil  as  far 
back  as  1864,  and  even  a  few  years  earlier  Virchow  had  described  a  multiple 
neuritis  occurring  together  with  leprosy.  Going  back  still  further,  the  histor- 
ical investigations  have  led  to  the  discovery  that  Dr.  Jackson,  of  Boston,  had 
recognized  peripheral  multiple  neuritis  as  early  as  1822  ;  but  all  these  re- 
searches, even  including  those  of  Duchenne,  did  not  help  to  place  the  knowl- 
edge of  multiple  neuritis  upon  a  firm  basis.  The  subject  was  revived  by  Jof- 
froy,  in  1879,  and  Leyden,  in  1880,  and  since  that  time  innumerable  articles 
have  appeared  by  writers  of  almost  every  nationality. 

Comparatively  few  writers,  however,  have  had  much  to  say  regarding 
multiple  neuritis  occurring  in  children.  Diphtheritic  paralysis,  to  be  sure,  was 
discussed  clinically  long  before  it  was  known  that  it  was  due  to  peripheral 
neuritis.  Multiple  neuritis  is  not  so  frequent  in  children  as  in  adults,  for  the 
simple  reason  that  the  causes  leading  to  it  are  not  as  powerful  in  early  life  as 
later  on ;  and  yet  cases  are  common  enough  to  necessitate  a  discussion  of 
them  in  this  book. 

Symptoms. — Whatever  the  cause  of  the  multiple  neuritis 
may  be  the  symptoms  are,  with  few  modifications,  practi- 


228 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


cally  the  same  in  all  cases.  Though  the  development  of  the 
various  symptoms  is  subject  to  great  variations,  the  symp- 
toms are  such  as  we  might  well  expect  on  physiological 
grounds  from  disease  of  the  peripheral  mixed  nerves.    The 


Fig.  56.— Young  Boy  with  Multiple  Neuritis,  showing  Double  "Wrist  Drop"  and 
Slight  "  Foot  Drop." 

most  characteristic  feature  of  multiple  neuritis  is  the  asso- 
ciation of  motor  paralysis  with  sensory  paralysis,  the  dis- 
tribution of  each  harmonizing  with  the  other  and  showing 
very  definite  anatomical  limits.  The  paralysis  is  of  the 
flaccid  order,  leads  at  an  early  date  to  atrophy  of  the  af- 


'MULTIPLE    NEURITIS.  229 

fected  muscles,  and  the  electrical  conditions  are  altered  so 
that  we  may  find  almost  every  possible  form  of  the  reac- 
tion of  degeneration  from  a  mere  loss  of  faradic  irritabil- 
ity to  an  absolute  loss  of  galvanic  response  on  the  part  of 
the  nerves  and  muscles.  The  distribution  of  the  paralysis 
is,  as  a  rule,  entirely  symmetrical,  and  may  affect  either 
the  upper  or  lower,  or  all  four  extremities;  it  may  involve 
every  part  of  all  the  extremities,  and  is  the  one  affection 
which,  perhaps  more  frequently  than  any  other,  leads  to 
a  complete  paralysis  of  every  limb  of  the  body.  It  is  one 
of  the  diseases,  therefore,  which  the  physician  should  bear 
in  mind  if  he  finds  a  patient  who  is  totally  paralyzed  with 
the  bare  exception  of  the  head  and  tongue,  the  jaws  and 
eyes  ;  sometimes  some  of  the  latter  parts  may  be  paralyzed 
as  well.  Certain  groups  of  muscles  are  more  readily  af- 
fected than  others  ;  thus  we  find  that  the  extensors  of  the 
wrist  and  the  extensors  of  the  feet  are  very  frequently  in- 
volved, and  at  an  early  period  of  the  disease  "  wrist-drop  " 
and  "  foot-drop  "  are  characteristic  phenomena  of  the  dis- 
ease. With  the  paralysis  sensory  symptoms  go  hand  in 
hand  ;  paralysis  associated  with  pain  may  be  noted  in  some 
cases  of  spinal-cord  disease,  as  in  the  acute  stages  of  a 
poliomyelitis,  but  the  persistence  of  pain  in  those  parts 
which  are  paralyzed,  and  along  the  nerve-tracts,  points  very 
strongly  to  a  multiple  neuritis.  Whenever  we  suspect  that 
a  paralysis  or  a  deformity  may  be  the  result  of  a  multiple 
neuritis,  the  previous  occurrence  of  a  painful  affection  and 
the  duration  of  the  same  must  be  determined  with  the 
greatest  care.  If  the  patient  is  seen  during  the  acute  stage  of 
the  disease  the  tracts  of  the  peripheral  nerves  will  be  found 
painful,  and  this  objective  proof  will  be  added  to  the  sub., 
jective  symptoms  which  the  patient  describes.  But  the  ab- 
sence of  pain,  as  evidenced  by  cases  reported  by  Barrs, 
need  not  militate  against  the  diagnosis. 

The  motor  and  sensory  paralysis  is  not  developed  as  rapidly  as  is  the 
case  in  central  affections.  There  is  a  premonitory  period  during  which 
numbness,  tingling,  sensations  of  heat  or  cold,  in  the  parts  to  be  affected 
later  on,  are  the  annoying  symptoms,  and  after  a  premonitory  period  that 
may  vary  from  a  few  days  to  a  few  months  the  development  of  the  paralysis 
may  be  acute,  subacute,  or  chronic.     In  the  majority  of  the  cases  it  is  sub- 


230  THE  NERVOUS  DISEASES   OF   CHILDREN. 

acute  or  chronic,  and  it  often  requires  a  period  varying  between  one  and  two 
weeks  until  the  weakness  of  the  parts  has  advanced  far  enough  to  be  con- 
sidered an  actual  paralysis.  The  onset  is  marked  occasionally  by  the  oc- 
currence of  fever,  and  if  the  disease  is  due  to  any  toxic  agent  that  has  con- 
stitutional effects  the  beginning  may  be  signalized  by  convulsions  as  well  as 
by  rigors.  Under  the  latter  circumstances  the  symptoms  may  very  closely 
resemble  those  of  an  acute  poliomyelitis,  but  it  is  rare,  after  all,  to  find  as 
stormy  an  onset  in  cases  of  multiple  neuritis  as  in  spinal  infantile  paralysis. 

Before  the  paralysis  becomes  absolute,  if  it  ever  reaches 
that  stage,  tremor  of  the  parts  and  inco-ordination  may  be 
associated  with  loss  of  power  and  loss  of  sensation.  The 
patient  may  be  able  to  stand  or  walk,  but  does  so  imper- 
fectly, sways  with  the  body  when  attempting  to  stand,  and 
may  fall  if  the  eyes  are  closed.  The  reflexes  connected 
with  the  parts  affected  by  the  paralysis  are  almost  inva- 
riably diminished  or  lost ;  this  is  particularly  true  of  the 
knee-jerk,  since  the  trouble  so  often  begins  in  and  involves 
the  legs  to  a  greater  extent  than  any  other  part  of  the 
body.  The  reflex  is  often  absent  before  the  paralysis  is 
developed.  An  exception  to  the  rule  of  loss  of  knee-jerk 
has  been  reported  by  Striimpell.  A  few  years  ago  he  de- 
scribed a  case  in  which  the  reflexes  were  increased,  and 
this  increase  he  attributed  to  irritation  of  the  afferent  por- 
tion of  the  reflex  arc. 

The  more  distal  parts  of  the  extremities  are,  as  a  rule, 
the  first  to  be  affected.  In  the  lower  extremities  the  ear- 
lier stages  of  the  disease  are  marked  by  an  inability  to 
raise  the  toes  while  the  heel  remains  on  the  ground,  show- 
ing a  weakness  of  the  extensors  of  the  toes,  and  implying 
a  loss  of  function  of  the  branches  of  the  anterior  tibial 
nerve.  It  is  a  curious  fact  that  the  muscles  supplied  by 
the  anterior  tibial  nerves  are  not  only  most  frequently  af- 
fected in  multiple  neuritis,  but  they  also  innervate  the  re- 
gion which  is  frequently  involved  in  poliomyelitis.  In  the 
upper  extremities  the  extensors  in  the  forearm  are  affected 
in  the  earliest  stages  of  the  disease,  and  from  these  parts 
the  paralysis  may  extend  upward  in  both  extremities  until 
the  greater  number  of  the  muscles  are  affected  or  until  total 
paralysis  of  all  the  extremities  is  established.  The  thenar 
and  hypothenar  groups  are  not  affected  at  the  outset.     It 


MULTIPLE    NEURITIS.  23  I 

will  be  seen  from  this  that  the  musculo-spiral  nerve  in  the 
upper  extremity,  and  its  homologue,  the  peroneal  nerve,  in 
the  lower  extremity,  are  the  nerves  first  attacked  by  the 
disease  in  a  large  number  of  the  cases.  This  peculiar  pre- 
disposition of  these  nerves  to  the  disease  cannot  be  ex- 
plained on  the  ground  of  anatomical  position,  and  for  the 
present  is  as  inexplicable  as  are  the  facts  of  elective  affin- 
ities in  so  many  other  diseases. 

In  some  cases  the  nerves  supplying  the  trunk  muscles, 
including  the  diaphragm,  may  be  affected,  but  this  is,  after 
all,  a  very  great  rarity.  A  paralysis  of  the  vocal  cords 
due  to  multiple  neuritis  is  rare  enough,  as  is  also  an  involve- 
ment of  the  facial  nerve  and  of  the  hypoglossal.  I  have, 
however,  seen  a  number  of  cases,  particularly  after  diph- 
theria, in  which  the  ocular  muscles,  the  vocal  cords,  and  the 
muscles  of  deglutition  were  paralyzed. 

The  paralysis,  although  it  may  involve  a  number  of  groups  of  muscles, 
does  not  affect  all  equally  ;  those  less  affected  may  undergo  contracture,  such 
contractures  occurring  quite  as  often  in  the  lower  limbs  as  in  the  upper 
extremities.  Deformities  quite  as  serious  and  quite  as  persistent  as  those  in 
poliomyelitis  may  therefore  result  from  a  chronic  multiple  neuritis. 

As  regards  the  sensory  symptoms  it  is  important  to 
note  that  every  form  of  sensation  is  about  equally  impaired  ; 
touch,  pain,  temperature,  and  muscular  sense  may  suffer 
equally  except  in  the  earlier  stages,  in  which  a  hyper- 
esthesia is  a  more  common  than  hypsesthesia.  In  a  few 
instances  the  pain  sensibility  may  be  diminished  while  the 
sensation  of  touch  remains,  and  even  if  the  sensibility  to 
pain  is  intact  the  conduction  of  pain  impressions  may  be 
very  much  delayed.  While  pain  and  paralysis  are  the  dis- 
tinguishing symptoms  of  a  multiple  neuritis  it  should  be 
remembered  that  in  not  a  few  instances  one  or  the  other 
may  be  absent,  and  in  some  cases  pain  may  never  have  been 
present  to  any  marked  degree,  or  may  have  lasted  for  so 
short  a  time  as  compared  with  the  paralysis  that  the  pa- 
tient who  is  examined  at  a  later  stage  of  the  disease  fails  to 
remember  that  pain  was  ever  present.  The  electrical  re- 
actions may  exhibit  every  form  of  change  from  a  very 
slight  diminution   of  faradic  and  galvanic  response   to   a 


232  THE  NERVOUS  DISEASES   OF   CHILDREN. 

partial  or  complete  reaction  of  degeneration.  Atrophy  is 
also  a  prominent  and  early  symptom.  The  entire  absence 
of  bladder  and  rectum  symptoms  is  of  great  importance  in 
the  diagnosis  of  multiple  neuritis.  Exceptions  to  this  rule 
have  been  recorded  by  Oppenheim. 

Vasomotor  changes  in  the  paralyzed  parts  constitute  a 
frequent  symptom  of  multiple  neuritis.  QEdema  and  glos- 
siness of  the  skin,  which  are  observed  in  the  majority  of  the 
cases  of  peripheral  nerve  disease,  are  present  in  many  cases 
of  multiple  neuritis.  If  the  hand  is  involved  there  is  a  pe- 
culiar tapering  of  the  fingers,  which  is  quite  characteristic 
of  disease  of  the  peripheral  nerves. 

The  course  of  the  disease  will  vary  very  much  according 
to  the  intensity  of  the  cause.  As  a  rule  the  symptoms  in- 
crease during  the  first  four  to  six  weeks  and  then  diminish. 
While  the  disease  is  increasing  sensory  symptoms  are  par- 
ticularly prominent.  •  As  soon  as  the  acme  of  the  disease 
has  been  reached  these  symptoms  become  less  troublesome. 
The  motor  paralysis  is  recovered  from  much  less  quickly 
than  are  the  disturbances  of  sensation.  This  is  due  not 
only  to  the  actual  loss  of  innervating  power  but  to  the 
atrophy  of  the  muscles  and  to  the  contractures  which  have 
been  formed.  But  even  in  severe  cases  a  favorable  change 
sets  in  sooner  or  later.  The  atrophy  diminishes  and  the 
strength  of  the  paralyzed  limbs  slowly  increases  until  com- 
plete recovery  is  established. 

In  some  instances  the  onset  of  the  disease  is  a  very  sudden  one.  The 
paralysis  spreads  rapidly  and  may  resemble  the  course  of  Landry's  paralysis  ; 
but  the  fact  that  in  multiple  neuritis  the  trunk  muscles  generally  escape  and 
that  the  paralysis  jumps  from  the  lower  extremities  to  the  upper  without  in- 
volving the  trunk,  will  help  to  differentiate  it  from  an  acute  ascending  palsy. 
While  we  have  good  reason  to  expect  every  case  of  multiple  neuritis  in  a 
child  to  lead  to  recovery,  death  may  result  in  consequence  of  complicating 
conditions,  such  as  paralysis  of  respiratory  muscles,  or  of  the  heart  from 
disease  of  the  vagus.  Bronchitis  and  pneumonia  are  complications  very 
much  to  be  feared  in  children  who  have  any  form  of  multiple  neuritis.  Cir- 
rhosis of  the  liver  and  gross  cerebral  disturbances,  due  to  alcoholism,  which 
are  common  causes  of  death  in  cases  of  multiple  neuritis  in  the  adult,  do  not 
play  a  similar  role  in  children.  On  the  other  hand,  the  involvement  of  the 
kidneys  in  cases  of  diphtheritic  palsy  is  a  frequent  cause  of  death.  The 
psychoses  described  in  connection  with  the  multiple  neuritis  of  adults  are  not 
met  with  in  children. 


MULTIPLE  NEURITIS.  233 

Multiple  neuritis  may  result  from  a  number  of  different 
causes:  I.  Toxic  forms,  including  those  due  to  metallic 
and  non-metallic  poisons.  Among  the  former  lead,  arsenic, 
and  alcohol  are  the  most  potent;  and  in  rare  instances 
mercury,  carbonic  oxide,  and  phosphorus  may  bring  on  a 
neuritis.  In  a  very  careful  article  in  Keating's  "  Encyclo- 
paedia," Dr.  Putnam,  of  Boston,  has  discussed  the  palsies  of 
children  due  to  lead  and  arsenic.  He  finds  that  poisoning 
by  arsenic  occurs  most  frequently  through  the  mixtures 
used  to  destroy  vermin,  such  as  "  Rough  on  Rats," *"  Paris 
Green,"  "German  Fly-paper,"  and  the  like.  Furthermore, 
arsenic  is  contained  in  many  of  the  coverings  used  for 
books,  toys,  confectionery,  labels,  etc.,  as  well  as  for  arti- 
ficial flowers.  The  same  author  also  believes  that  wall-paper 
and  some  special  forms  of  carpet  may  be  the  source  of  poi- 
soning by  arsenic.  The  arsenic  is  supposed  to  be  commu- 
nicated to  the  child  in  the  form  of  dust  distributed  in  the 
currents  of  air,  or  in  the  form  of  some  volatile  compound 
which  may  be  formed  in  a  room  which  contains  arsenious 
wall-paper. 

That  some  cases  occur  from  the  continued  administration  of  arsenic  in 
diseases  like  chorea  there  is  very  little  doubt,  and  it  is  well  to  watch  children 
carefully,  while  they  are  undergoing  this  form  of  treatment.*  As  for  lead,  it 
may  enter  the  system  from  glazed  cards,  acid  fruits  and  vegetables  put  up  in 
tins,  also  from  chocolate  and  other  articles  wrapped  in  tinfoil.  Lead  also 
enters  largely  into  the  composition  of  rubber,  and  has  been  found  in  the  rub- 
ber nipples  of  nursing-bottles.  Various  kinds  of  cakes  and  candies  are 
treated  with  lead,  and  prove  a  prolific  source  of  poisoning  in  children  ;  but 
children  as  well  as  adults  are  exposed  to  lead-poisoning  very  much  more  fre- 
quently through  drinking-water  than  in  any  other  way.  This  is  more  com- 
mon in  country  districts,  where  lead  pipes  are  connected  with  pumps,  or 
where  lead  pipes  are  used  to  convey  water  from  wells  or  springs  at  varying 
distances  to  the  house.  In  the  large  cities  this  special  source  of  lead-poison- 
ing is  extremely  rare. 

Alcoholic  poisoning  in  children  would  naturally  be  con- 
sidered a  rare  condition,  and  so  it  fortunately  is  :  but 
among  the  ignorant  classes  of  every  large  population  par- 
ents will  be  found  who  take  special  delight  in  the  fact  that 
their  child   can   partake    of  everything,  including  beer  or 

*  I  have  seen  one  case  of  neuritis  in  the  adult  after  the  use  of  an  arsenic  paste  over 
a  large  denuded  surface. 


234  THE  NERVOUS  DISEASES   OF   CHILDREN. 

whiskey.  A  case  of  alcoholic  neuritis  in  a  child  five  years 
old  was  reported  years  ago  from  Striimpell's  clinic.  The 
child  was  raised  in  the  saloon  of  its  father,  and  not  only 
drank  considerable  wine,  but  also  two  quarts  of  beer, 
every  day.  The  father  is  quoted  as  saying  that  "  the  child 
was  always  thirsty,  and  of  course  we  could  not  give  it 
water." 

2.  The  toxsemic  forms  of  multiple  neuritis  are  far  more 
important — at  least  in  children.  Among  these  we  class,  first 
of  all,  the  septic  forms  of  multiple  neuritis  which  may  occur 
in  children  as  in  adults  after  injury  of  any  sort.  The  inva- 
sion of  micro-organisms  is  unquestionably  the  direct  cause 
of  the  neuritis. 

A  similar  invasion  of  specific  organisms  would  account  for  the  neuritis  of 
leprosy  and  of  beriberi,  but  these  are  so  rare  in  this  country,  and  particularly 
rare  in  children,  that  we  need  not  make  any  further  mention  of  them. 

Malarial  neuritis  is  of  great  interest  to  us,  for  though  it 
has  been  described  chiefly  by  authors  in  Eastern  countries, 
it  is  not  unknown  in  these  parts.  I  have  seen  several  cases 
of  this  description  in  children  who  were  brought  to  my 
clinic  from  the  swampy  parts  of  Long  Island. 

Dr.  Browning,  of  Brooklyn,  has  reported  several  such 
cases,  which  were  supposed  to  be  cases  of  poliomyelitis. 
These  cases  resemble  poliomyelitis  in  their  onset  and  devel- 
opment, and  may  be  easily  mistaken  for  it.  In  its  general 
symptoms  this  form  differs  but  very  little  from  other  cases 
of  multiple  neuritis  except  that  it  is  associated  much  more 
frequently  with  the  intermittent  form  of  fever,  that  the  dis- 
ease itself  undergoes  marked  remissions,  and  that  the  en- 
largement of  the  spleen  and  examination  of  the  blood  are 
likely  to  give  positive  proof  of  the  origin  of  the  neuritis. 
Recovery  is  a  little  more  rapid,  too,  in  these  cases  than  in 
those  due  to  other  causes. 

Among  the  toxaemic  conditions  which  give  rise  to  mul- 
tiple neuritis  we  must  include  those  blood  states  which  fol- 
low upon  the  various  acute  infectious  diseases  (diphtheria, 
small-pox,  typhoid  fever,  tuberculosis,  and  also  syphilis).  As 
the  nerve  affection  is  developed  a  long  time  after  the  initial 
infection  it  is  probable,  in  the  light  of  recent  bacteriological 


MULTIPLE  NEURITIS.  235 

researches,  that  some  chemical  product  of  the  micro-organ- 
isms, and  not  the  bacteria  themselves,  are  the  direct  cause 
of  the  neuritis.  With  the  exception  of  diptheritic  multiple 
neuritis  (p.  240)  all  the  forms  just  mentioned  are  compara- 
tively rare  in  children. 

In  obedience  to  the  ordinary  belief,  we  must  concede  the 
existence  of  the  rheumatic  forms  of  multiple  neuritis;  but  I 
am  free  to  confess  that  I  have  seen  only  a  very  few  cases  of 
multiple  neuritis  in  children  which  could  with  some  degree 
of  probability  be  referred  to  rheumatic  causes.  In  cases  of 
articular  rheumatism  in  children  the  nerves  are  sometimes 
involved,  as  is  proved  by  pains  radiating  down  the  limb 
through  special  nerve-trunks.  Refrigeration  neuritis  oc- 
curs in  children.  In  one  instance  this  was  due  to  ice-cold 
baths  which  a  mother  gave  to  her  child  daily  all  the  year 
around.  Now  and  then  cases  come  under  one's  notice  of 
severe  forms  of  polyneuritis  in  which  the  true  cause  cannot 
be  ascertained.  Specific  and  tuberculous  forms  of  multiple 
neuritis  are  rare  indeed. 

Pathological  Anatomy. — It  is  natural  to  infer  that 
the  changes  are  very  much  the  same  as  those  to  be  found 
in  cases  of  ordinary  simple  neuritis  (see  page  209).  In  mul- 
tiple neuritis  the  changes  are  chiefly  of  a  parenchymatous 
order,  or  interstitial  in  character;  the  perineurium  is  not 
often  involved.  In  some  cases,  however,  the  nerve  is  swol- 
len, the  nerve-sheath  is  hyperasmic,  of  a  deep  purplish 
color,  and,  according  to  Eichhorst,  may  be  covered  with 
minute  hemorrhages.  There  may  be  some  slight  changes 
also  in  the  consistence  of  the  nerve.  All  these  conditions, 
and  the  appearances  of  degeneration,  will  vary  according 
to  the  stage  of  the  disease  at  the  time  of  examination.  In 
the  earlier  stages  we  often  find  the  nuclei  of  the  fibres  en- 
larged, a  proliferation  of  the  connective  tissue  between 
the  nerve-elements,  and  numerous  round  or  spindle-shaped 
cells  with  myeline  granules  near  the  sheath  of  the  nerve. 
If  the  specimen  is  examined  at  a  later  period  of  the  disease 
the  acute  condition  will  have  passed  and  the  nerve  presents 
the  simple  appearance  of  subacute  or  chronic  degeneration 
of  nerve-fibres.     (Fig.  57.) 

In  some  cases  the  muscular  tissue  is  slightly  involved, 


236 


THE   NERVOUS  DISEASES   OF   CHILDREN. 


the  fibres  are  smaller  and  paler,  the  transverse  striation  is 
less  distinct  than  under  normal  conditions,  while  the  nuclei 
of  the  fibres  and  of  the  interstitial  tissues  may  be  increased. 
The  changes  in  the  muscle  may  be  both  parenchymatous 
and  interstitial. 

In  considering  the  pathology  of  multiple  neuritis,  the 
most  striking  fact  is  the  peculiar  action  of  toxic  agents  upon 
the  peripheral  nerves,  without  affecting  the  spinal  centres. 
The  farther  away  the  nerve-fibre  is  from  the  mother-cell 
the  less  able  it  is  to  resist  morbid  agencies ;  for  this  reason 
the  more  distal  parts  of  the  peripheral  nerves  are  affected 
at  an  earlier  period  in  multiple  neuritis  than  are  the  parts 
of  the  nerve  nearer 
the  spinal  centres. 

This  argument  is 
strengthened  by  the  re- 
cent conceptions  of  the 
close  relationship  between 
the  ganglion-cell  and  the 
peripheral  nerve -fibre  as 
parts  of  a  neuron.  More- 
over,  recent  researches 
have  shown  that  the  same 
poison  which  often  affects  FlG  57>_Chronic  Interstitial  Neuritis  with  Atrophy  of 
the  peripheral  nerve-fibre  SOme  Nerve-fibres.  (Ziegler.)  a,  cross-sections  of 
may  occasionally  involve  normal  nerve-fibres  ;  b,  cross-section  of  thin,  but  nor- 
the  eanehon-cell.  Stieglitz  mali  nerve-fibres  ;  c,  endoneurium  ;  d,  cellular  in- 
v        '  a  a;„  +n  k^  «-v,o        filtration  and  proliferation  of  connective-tissue  strands 

has  proved  this  to  be  the  . v  . 

r  .     .        .  and   blood-vessels  ;  /,  thickened   endoneurium  with 

case  in  lead-poisoning.  He       smaU  empfy  nerve.spaces  and  some  normal  thin  nerve- 
found  a  distinct  poliomy-        fibres .  g<  longitudinal  section  of  a  blood-vessel, 
elitis  in  animals  compelled 

to  inhale  lead  salts.  Oppenheim  has  described  slight  changes  in  the  spinal 
cord  in  cases  of  alcoholic  neuritis.  Recent  investigators  have  found  changes 
in  the  spinal  and  cranial  nerve  roots,  and  even  in  the  nerve  elements  of  the 
brain  (Marinesco,  Schlesinger).  It  is  certain  that  in  man  lead  may  cause 
disease  of  the  peripheral  nerves. 

Diagnosis. — With  our  present  knowledge  of  the  charac- 
teristic symptoms  of  multiple  neuritis  there  should  be  no 
difficulty  in  differentiating  between  the  various  forms  of 
this  disease  and  other  diseases  with  somewhat  similar 
symptoms.  The  chief  characteristic  of  these  peripheral 
nerve   diseases   is   the  association  of  sensory  with   motor 


MULTIPLE   NEURITIS.  237 

symptoms,  the  distribution  of  both  pointing  to  an  in- 
volvement of  the  same  nerve-areas.  Persistent  sensitive- 
ness of  the  nerve-traets  and  subjective  pain  in  the  course  of 
the  peripheral  nerves  help  to  corroborate  the  diagnosis.  It 
is  easy  to  mistake  multiple  neuritis  for  poliomyelitis,  and 
until  recent  years  this  mistake  was  made  by  the  ablest  diag- 
nosticians; but  in  poliomyelitis  the  initial  symptoms  are 
of  a  rather  more  violent  character,  and  the  entire  central 
nervous  system  often  shows  the  influences  of  the  disease 
very  much  more  than  it  does  in  multiple  neuritis ;  the 
paralysis  is  more  symmetrical  in  neuritis  than  in  poliomye- 
litis. 

In  poliomyelitis  there  is  no  pain  along  the  nerve-tracts  and  these  nerve- 
tracts  are  not  sensitive,  but  vague  pains  are  very  often  present  in  the  first 
week  or  two  of  poliomyelitis.  [  have  the  record  of  a  child,  two  years  old, 
whom  I  saw  two  days  after  the  onset  of  the  paralysis,  in  whom  the  entire 
limb  was  so  painful  that  the  child  would  scream  violently  if  the  limb  was 
suddenly  touched,  and  this  was  not  due  to  the  fear  of  examination.  The  pain 
was  not  distributed  along  any  nerve-paths,  and  I  did  not  hesitate  to  make 
the  diagnosis  of  poliomyelitis,  which  proved  to  be  the  correct  one  by  the 
subsequent  course  of  the  disease.  The  electrical  reactions  are  very  much 
the  same  in  both  disorders. 

The  difficulties  of  this  problem  are  increased  by  the  fact  that,  as  Gowers 
has  reported,  cases  occur  in  which  a  typical  anterior  poliomyelitis  is  compli- 
cated by  a  peripheral  neuritis.  In  spite  of  all  efforts  at  differentiation  cases 
will  occur  every  now  and  then  in  which  it  will  be  impossible  to  make  an  ac- 
curate diagnosis  at  the  start,  and  the  physician  must  watch  the  course  of  the 
disease  before  coming  to  a  definite  conclusion. 

From  pachymeningitis,  which  is  rare  in  children,  the  disease  may  be  dif- 
ferentiated more  easily,  since  in  the  former  the  legs  are  rarely  affected,  the 
anaesthesia  is  apt  to  spread  to  the  upper  limbs  and  to  the  trunk  of  the  body, 
and  there  is  no  distinct  sensitiveness  to  pressure  on  the  part  of  the  larger 
nerve-trunks,  except  late  in  the  disease,  and  the  atrophy  is  restricted  to 
muscles  innervated  from  the  diseased  spinal  segment. 

Landry's  paralysis  is  to  be  recognized  by  the  rapidly  ascending  palsy  pro- 
ceeding from  segment  to  segment,  involving  the  abdominal  and  thoracic 
muscles,  and  not  skipping  from  the  lower  to  the  upper  extremities,  as  is  the 
case  in  poliomyelitis.  The  differentiation  between  the  ataxic  forms  of 
multiple  neuritis  or  the  so-called  pseudo-tabes  from  true  tabes  need  hardly 
be  considered  in  the  case  of  children,  as  in  them  true  tabes  is  a  disease  of 
exceeding  rarity.  But  it  may  be  well  to  bear  in  mind  that  the  symptoms  of 
hereditary  ataxia  (Friedreich's  disease),  including  the  loss  of  knee  -  jerk, 
ataxic  gait,  weakness,  and  awkwardness  in  walking,  may  bear  a  superficial 
resemblance  to  polyneuritis  ;  but  the  gradual  development  of  the  symptoms  in 


238  THE  NERVOUS  DISEASES   OF   CHILDREA. 

this  hereditary  disease,  the  occurrence  in  various  members  of  the  same  family, 
the  peculiar  appearance  of  the  face,  the  disturbance  of  speech,  the  mental 
peculiarities,  will  render  the  diagnosis  positive  enough. 

As  children  are  subject  to  hysterical  palsies  the  question  of  differential 
diagnosis  may  occasionally  arise,  but  hysterical  palsy  is  not  characterized  by 
the  loss  of  reflexes  ;  nor  is  the  ataxia,  if  present,  like  that  of  polyneuritis  ;  and, 
moreover,  the  anaesthesia  accompanying  the  palsy  in  hysterical  cases  is  of 
the  regional  order,  and  not  in  keeping  with  the  anatomical  distribution  of  the 
nerves.  If  there  is  any  reason  to  suspect  myelitis,  the  presence  or  absence 
of  bladder  and  rectal  symptoms,  the  development  or  non-development  of  bed- 
sores will  weigh  heavily  for  or  against  the  diagnosis  of  cord  disease. 

Trichinosis  must  be  suspected  in  cases  of  great  painfulness  of  the  ex- 
tremities, but  the  other  attendant  symptoms  (gastric  disturbances)  and  the 
excessive  swelling  of  the  muscles  will  help  to  clear  up  the  diagnosis. 

The  recognition  of  the  cause  of  multiple  neuritis  is  an  important  point 
in  the  diagnosis  of  the  disease.  In  this  country  the  disease  in  children  is 
developed  most  frequently  after  an  acute  infection,  either  by  poisoning  or 
by  the  toxic  products  of  some  acute  infectious  disease,  such  as  diphtheria, 
variola,  and  typhoid  fever.  Next  in  frequency  I  would  place  the  probability 
of  a  malarial  origin,  and  last,  the  possibility  of  tubercular,  syphilitic,  or  alco- 
holic infection. 

Treatment. — First  of  all  we  must  determine  the  pri- 
mary cause  of  the  disease,  and,  if  possible,  the  deleterious 
agent  must  be  removed  or  its  influence  arrested.  If  the  child 
lives  in  a  malarial  district  it  should  be  taken  to  a  place 
where  there  is  no  danger  from  further  malarial  infection. 
If  there  is  any  suspicion  of  alcoholic,  or  of  lead,  or  of  arseni- 
cal poisoning,  the  sources  from  which  such  poisons  have 
emanated  must  be  absolutely  cut  off.  If  some  acute  infec- 
tious disease  has  preceded,  we  can,  of  course,  do  nothing  to 
prevent  the  natural  course  of  the  neuritis,  but  by  improving 
the  child's  general  health  will  enable  it  to  conquer  the  dis- 
ease much  more  rapidly.  For  the  relief  of  pain,  which  is 
by  far  the  most  annoying  symptom  at  the  beginning  of  the 
disease,  the  application  of  heat  is  the  best  remedy  we  have. 
If  heat  is  applied  at  later  stages  of  the  disease,  when  con- 
siderable anaesthesia  may  be  present,  it  is  of  the  greatest 
importance  to  watch  the  condition  of  the  skin,  or  else  seri- 
ous burns  and  ulcerations  may  result.  It  is  on  this  account* 
also,  that  the  use  of  the  actual  cautery  and  of  other  forms 
of  counter-irritation  is  not  to  be  recommended.  Prolonged 
warm  baths  will  often  help  to  alleviate  the  suffering,  and 


MULTIPLE    NEURITIS.  239 

will  contribute  largely  to  the  comfort  of  the  patient.  If 
the  pain  is  so  intense  that  it  cannot  be  relieved  by  these 
simple  measures  it  will  be  well  to  give  small  doses  of  mor- 
phia, codeia,  or  rectal  injections  of  chloral  hydrate. 

There  is  no  drug  which  has  any  specific  action  upon 
multiple  neuritis.  I  have  given  the  salicylates  invariably, 
but  am  not  convinced  that  they  have  done  much  good. 
Mercurials  have  been  praised  by  some,  particularly  in  cases 
in  which  the  nerve-sheath  is  involved  rather  than  in  the 
parenchymatous  form,  yet  I  have  not  been  able  to  persuade 
myself  of  the  benefits  to  be  derived  from  mercurials,  even 
in  many  cases  of  multiple  neuritis  in  the  adult ;  but  if  there 
is  any  good  reason  for  the  employment  of  mercury  let  it  be 
given  in  the  form  of  inunctions  of  unguentum  hydrargyri 
or  of  a  ten  or  twelve  per  cent,  oleate  of  mercury. 

The  administration  of  mercury,  either  by  the  mouth  or 
in-the  form  of  hypodermic  injections,  is  to  be  avoided  in  chil- 
dren, if  possible.  Iodide  of  potash  or  iodide  of  sodium  has 
little  or  no  influence  upon  the  disease ;  arsenic,  which  has 
gained  such  popularity  in  every  form  of  chronic  nerve  dis- 
ease, is  scarcely  a  safe  remedy  to  use,  for  in  small  doses  it 
will  have  no  effect,  and  if  given  in  large  doses  there  is  a  de- 
cided danger  of  increasing  the  neuritis.  We  are  compelled, 
therefore,  in  all  cases  of  multiple  neuritis  in  children,  par- 
ticularly in  those  occurring  after  diphtheria  and  other  acute 
infectious  diseases,  to  resort  to  general  tonic  remedies. 
Among  these  none  is  better  than  cod-liver  oil,  and  next  in 
order,  though  far  inferior  to  it,  are  iron,  quinine,  and 
strychnia  in  very  small  doses.  In  all  cases  of  malarial 
neuritis,  quinine  should  be  given  in  the  same  doses  that 
would  be  administered  to  combat  other  symptoms  of  mala- 
rial poisoning.  A  word  of  caution  should  be  added  with 
reference  to  the  use  of  anodynes.  In  children  the  careless 
exhibition  of  morphia  may  result  in  the  formation  of  the 
morphia  habit,  and  morphia  itself,  if  given  in  any  consid- 
erable quantities,  may  help  to  intensify  rather  than  to 
diminish  the  symptoms. 

If  insomnia  is  persistent  in  consequence  of  the  pain  in 
the  initial  stages,  one  of  the  many  hypnotics,  such  as  chlor- 
alamid,  trional,  or  veronal,  in  doses  varying  between  five 


240  THE   NERVOUS  DISEASES    OF   CHILDREN. 

and  fifteen  grains,  according  to  the  age  of  the  child,  should 
be  given  a  trial ;  if  they  do  not  produce  sleep,  codeia  in 
doses  of  one-sixth  to  one-half  of  a  grain  may  be  adminis- 
tered. 

After  the  first  two  or  three  weeks  of  the  disease  the 
paralytic  symptoms  become  rather  distinct.  In  all  those 
cases  in  which  there  is  considerable  paralysis  with  atrophy 
the  use  of  the  galvanic  current,  both  as  a  sedative  and  as  a 
means  of  exercising  the  paralyzed  parts  should  be  em- 
ployed. The  faradic  current  will  be  of  little  service  if  it 
fails  to  produce  contractions,  and  as  it  has  a  distinct  irritat- 
ing effect  upon  the  sensory  filaments  in  the  skin  it  should 
not  be  applied.  When  all  pain  has  disappeared  and  the 
progress  of  the  disease  has  been  stayed,  light  massage  can 
be  employed  to  advantage  ;  it  is  specially  to  be  recom- 
mended at  the  time  when  contractures  are  about  to  be  de- 
veloped, or  when  the  atrophy  of  the  muscles  is  on  the  in- 
crease. If  permanent  contractures  result  from  multiple 
neuritis  surgical  interference  may  be  necessary,  but  as 
the  disease  generally  leads  to  spontaneous  recovery,  every 
possible  means  of  bringing  this  about  should  be  employed 
before  the  child  is  given  over  to  the  surgeon  ;  yet  I  can 
see  no  harm  in  having  a  simple  tenotomy  performed  if  tha.t 
will  enable  the  child  to  bring  its  feet  to  the  ground,  or  to 
walk  before  complete  recovery  from  all  the  symptoms  has 
set  in.  In  some  of  the  cases  some  simple  orthopedic  ap- 
paratus will  help  the  child  to  learn  to  walk,  and  will  enable 
it  to  use  muscles  which  cannot  be  depended  upon  to  sup- 
port the  body  ;  but  there  is  also  a  danger  in  allowing  chil- 
dren to  wear  an  apparatus  too  long  a  time,  as  they  are  very 
prone  to  depend  entirely  upon  such  artificial  support,  and, 
as  a  rule,  lack  the  energy  to  exercise  weakened  muscles. 

DIPHTHERITIC   PARALYSIS. 

Paralysis  after  diphtheria  is  far  less  frequent  in  this  city 
than  it  was  before  the  use  of  the  Diphtheria  Anti-toxin,  and 
less  frequent  (relatively)  in  children  than  in  the  adult. 
In  earliest  infancy,  say  up  to  the  age  of  two  years,  diph- 
theritic   palsy   is    extremely    rare.       It    bears    no   absolute 


MULTIPLE    NEURITIS.  24 1 

relation  to  the  severity  of  the  diphtheritic  infection.  I  have 
often  seen  typical  diphtheritic  palsy  set  in  after  an  infec- 
tion so  slight  that  it  was  considered  to  be  nothing  but  a 
mild  sore  throat ;  but,  on  the  other  hand,  it  also  occurs  in 
connection  with  severe  diphtheritic  infection,  and  is  fre- 
quently associated  with  other  complicating  diseases,  such 
as  nephritis  and  endocarditis,  following  upon  the  original 
disease.  The  previous  health  of  the  child  has  no  distinct 
bearing  upon  the  development  of  the  palsy,  strong  and 
weak  children  being  affected  with  equal  frequency.  I  have 
seen  the  paralysis  developed  within  the  first  week,  while  the 
throat  was  still  covered  with  diphtheritic  membrane,  but 
the  majority  of  cases  do  not  occur  until  two,  three,  or  four 
weeks  after  the  termination  of  the  disease. 

Diphtheritic  palsy  differs  from  other  forms  of  multiple 
neuritis  in  the  order  in  which  the  various  parts  become 
paralyzed.  The  palate  is  generally  the  first,  often  the  only, 
part  affected.  Nasal  speech  and  regurgitation  of  liquids 
through  the  nose  are  evidences  of  paralysis  of  this  part,  and 
the  loss  of  function  can  be  determined  by  an  inspection  of 
the  mouth  and  throat.  Loss  of  accommodation  is  next  most 
frequent.  If  the  paralysis  extends  further,  the  upper  and  i 
lower  extremities  are  apt  to  be  involved.  At  first  a  mere  ! 
weakness  is  noticeable,  associated  with  tingling  and  numb- 
ness of  the  parts ;  later  on  this  weakness  increases,  and 
develops  into  a  full-fledged  paralysis,  and  the  disturbance 
of  sensation  may  become  more  marked.  The  ocular  mus- 
cles show  a  peculiar  predisposition  to  diphtheritic  infec- 
tion ;  the  rectus  externus,  supplied  by  the  sixth  nerve,  is 
frequently,  and  often  singly,  affected,  and  the  oculo-motor 
nerves  are  also  involved  at  times,  but  a  complete  ocular 
motor  palsy  in  diphtheria  is  rare.  Ptosis  and  paralysis  of 
one  or  more  muscles  supplied  by  the  third  nerve  have  fre- 
quently been  noticed.  The  optic  nerve  fortunately  escapes, 
so  that  blindness  is  not  a  common  result  of  diphtheria,  un- 
less by  some  untoward  accident  the  diphtheritic  poison 
should  cause  ulceration  or  purulent  disease  of  the  eye,  with 
subsequent  loss  of  vision.  The  ciliary  muscle  is  often  par- 
alyzed ;  the  reaction  of  the  pupils  to  light  is  sluggish,  but 
the  contraction  during  accommodation  may  be  preserved, 


242  THE   NERVOUS  DISEASES   OF   CHILDREN. 

although  the  act  of  accommodation  itself  is  carried  out  im- 
perfectly. 

Paralysis  of  the  pharynx  may  occur,  but  is  not  frequent; 
the  larynx,  however,  often  comes  in  for  a  share  of  the  pa- 
ralysis ;  the  epiglottis  then  fails  to  perform  its  function,  and 
food  may  reach  the  larynx  and  cause  severe  fits  of  cough- 
ing. Hoarseness  and  imperfect  phonation  point  to  an  in- 
volvement of  the  muscles  of  the  larynx  and  of  the  vocal 
cords.  This  special  order  of  paralysis  is  generally  sym- 
metrical. 

The  reflexes  are  diminished  or  lost,  even  when  no  dis- 
tinct paralysis  of  the  adjacent  parts  exists  or  existed  (Bern- 
hardt). I  have  had  opportunity  to  examine  several  children 
whose  knee-jerks  were  absent  after  diphtheria,  but  who 
had  never  presented  any  paralytic  symptoms.  The  same 
condition  is  found  occasionally  after  scarlatina  and  typhoid 
fever. 

In  the  limbs  the  change  in  the  motor  and  sensory  functions  is  very  like 
that  of  other  cases  of  multiple  neuritis,  and  does  not,  thererefore,  require 
any  special  mention.  It  is  questionable  whether  cardiac  failure  coming  on 
after  diphtheria  is  due  to  a  neuritis  of  the  pneumogastric,  or  whether,  as 
Leyden  would  have  it,  it  is  due  to  a  degeneration  of  the  heart  muscle  itself. 
The  irregularities  of  respiration  associated  with  the  heart  symptoms  are  so 
characteristic  of  vagus  affections  that  it  seems  much  more  probable  to  attrib- 
ute the  loss  of  function  to  disease  of  the  nerve  rather  than  of  the  muscular 
tissue. 

Some  diphtheritic  palsies  occur  which  do  not  in  any  way  resemble  mul- 
tiple neuritis  ;  a  hemiplegia  may  follow  upon  diphtheria,  but  this  may  be  the 
result  of  a  vascular  lesion  so  common  in  connection  with  other  infectious  dis- 
eases of  childhood.  Cases  of  this  character  have  been  described  by  Mendel 
and  Bonath. 

Paralysis  of  the  masseters  has  been  reported  by  Grant  and  quoted  by 
Gowers.  As  the  paralysis  was  permanent  fourteen  months  after  the  disease 
Gowers  thinks  the  affection  due  to  an  acute  nuclear  inflammation.  A  degen- 
eration of  various  cranial  nerves  has  been  observed  to  come  on  after  a  con- 
siderable  lapse  of  time,  following  upon  diphtheria,  but  it  has  seemed  a  little 
doubtful  whether  such  paralysis  could  be  traced  directly  to  a  diphtheritic  poi- 
son. 

The  general  course  of  the  disease  leads  to  recovery.  It 
may  last  from  six  to  eight  weeks ;  the  cases  in  which  single 
nerves  only  are  affected  recover  more  rapidly  than  those 
with  multiple  nerve-lesions.      The  ocular  nerves  recover 


MULTIPLE    NEURITIS.  243 

very  much  more  quickly  than  the  nerves  of  the  lower  ex- 
tremities do.  If  there  is  paralysis  of  the  legs,  it  takes,  as  a 
rule,  from  four  to  six  months  before  the  symptoms  disap- 
pear. The  reflexes  are  late  in  returning,  and  in  most  cases 
cannot  be  elicited  until  some  weeks  after  all  other  symp- 
toms have  passed  away. 

If  death  ensues  during  diphtheritic  palsy,  it  is  either  due  to  some  compli- 
cating disease,  such  as  nephritis  or  pneumonia,  or  to  paralysis  of  the  heart 
or  respiratory  muscles.  Mere  exhaustion  from  inability  to  take  food  in  cases 
of  paralysis  of  the  pharynx  is  an  occasional  but  rare  cause  of  death. 

Pathological  Anatomy. — The  morbid  changes  are 
essentially  the  same  as  those  found  in  other  forms  of  multi- 
ple neuritis  ;  the  inflammatory  and  degenerative  changes  in 
the  nerves  are  in  these  cases  probably  the  result  of  micro- 
bic  poisoning,  or  the  effect  of  chemical  poisons  formed  by 
the  diphtheritic  micro-organisms  ;  but  bacteriological  re- 
searches have  not  yet  yielded  a  very  satisfactory  explana- 
tion of  all  the  changes  that  take  place  in  the  peripheral 
nerves.  The  rod-shaped  bacteria,  the  bacilli  of  Klebs  and 
Loerfler,  have  been  found  in  the  blood-vessels  of  the  nerve- 
centres,  and  other  micrococci  have  been  described  by  Oer- 
tel  as  occurring  in  these  vessels  ;  but  further  study  will  be 
needed  to  explain  the  origin  of  the  inflammatory  process  in 
the  nerves.  The  degeneration  is  found  in  the  nerve  supply- 
ing the  paralyzed  part ;  often  the  entire  nerve  is  affected,  at 
times  some  of  the  filaments  only.  In  others,  the  degenera- 
tion extends  to  the  anterior  or  even  the  posterior  roots. 
The  myelin  and  nerve-filaments  undergo  segmentation, 
the  nuclei  of  the  sheath  are  increased,  and  granular  corpus- 
cles mark  the  decay  of  nerve-tissue.  The  axis-cylinder 
remains  intact  until  the  destruction  of  the  medullary  sheath 
has  advanced  considerably,  and  both  these  parts  may  then 
be  entirely  destroyed.  The  interstitial  tissue  between  the 
nerve-fibres  is  but  little  affected,  thus  marking  the  condi- 
tion as  a  true  parenchymatous  degeneration.  The  nerves 
supplying  the  palate  are  most  frequently  and  most  severely 
affected,  but  the  same  changes  may  occur  in  the  peripheral 
nerves  of  the  extremities  in  all  the  ocular  nerves,  and  even 
the  phrenic  nerve  may  be  similarly  affected. 


244  THE  NERVOUS  DISEASES    OF   CHILDREN. 

In  diphtheritic  paralysis  the  muscular  tissue  is  more  distinctly  changed 
than  in  other  forms  of  paralysis  due  to  multiple  neuritis.  In  cases  of  long 
standing  the  muscular  fibre  of  the  palate,  for  instance,  is  found  to  have  under- 
gone granular  and  fatty  degeneration.  In  some  there  are  signs  of  parenchy- 
matous as  well  as  of  interstitial  inflammation,  and  every  possible  grade  may 
be  found  between  a  simple  inflammatory  condition  and  complete  degenera- 
tion of  the  muscular  fibres.  In  cases  in  which  the  disease  leads  to  implica- 
tion of  the  heart  muscle,  the  pallor  of  the  heart  after  death  is  the  external 
evidence  of  complete  degeneration  of  its  muscular  fibres. 

Much  has  been  made  of  changes  in  the  spinal  cord  in  cases  of  diphthe- 
ritic paralysis,  but  these  are  restricted  entirely  to  changes  of  the  ganglion  cells 
of  the  anterior  horns,  which  have  been  found  swollen,  homogeneous  in 
character,  and  with  processes  somewhat  altered  or  entirely  shrunken  ;  but  it 
is  doubtful  whether  these  changes  are  primary  or  secondary,  though  there  is 
no  good  reason  why  a  poison  which  acts  so  vigorously  upon  the  peripheral 
nerves  should  not  invade  the  various  tracts  of  the  spinal  cord. 

The  frequent  disease  of  the  palate  is  probably  due  to  the  proximity  of 
the  nerves  supplying  the  palate,  to  the  diphtheritic  membranes,  and  the  eas- 
ier invasion  into  these  nerves  of  the  micro-organisms  lodged  in  the  tonsils, 
or  in  the  back  of  the  throat.  And  yet  if  we  remember  that  a  considerable 
period  of  time  may  elapse  between  the  deposit  of  the  diphtheritic  membranes 
in  the  throat  and  the  appearance  of  the  palsy,  and  that  the  palsy  may  appear 
after  very  mild  throat  affections,  it  is  not  altogether  easy  to  explain  why  these 
nerves  should  be  so  frequently  the  seat  of  the  disturbance,  unless  we  suppose 
that  from  their  proximity  to  the  original  diphtheritic  lesion  the  nerve-tissue 
has  become  altered  and  thus  rendered  more  susceptible  to  the  diphtheritic 
microbes,  or  to  the  microbic  toxins.  The  poison  attacks  other  nerves  in 
the  course  of  time ;  the  peroneus,  the  musculo-spiral,  and  the  median  are  the 
most  susceptible  to  it. 

The  diagnosis  of  this  palsy  often  depends  upon  the 
history  of  preceding  diphtheria,  or  of  some  throat  affection, 
however  slight  it  may  have  been.  The  beginning  of  the 
palsy  in  the  palate,  and  gradually  spreading  to  the  lower 
and  upper  extremities,  will  at  once  suggest  the  probability 
of  diphtheritic  paralysis.  It  is  only  in  cases  in  which  the 
original  disease  has  been  overlooked  or  forgotten  that  an 
examination  of  the  patient  some  weeks  after  the  onset  of 
the  trouble  may,  through  the  atrophic  paralysis  and  the  ab- 
sence of  knee-jerk,  suggest  spinal  infantile  palsy  ;  but  a 
closer  examination  of  the  patient,  and  the  comparatively 
rapid  development  of  the  palsy,  with  its  tendency  to  recov- 
ery, will  place  the  diagnosis  beyond  doubt.  Diphtheritic 
palsy,  like  other  forms  of  paralysis  in   children,  may  con- 


•      MULTIPLE    NEURITIS.  245 

tinue  for  an  inordinate  length  of  time,  and  may  be  super- 
seded by  an  hysterical  palsy.  Thus  it  happens  not  infre- 
quently, as  in  the  daughter  of  a  clergyman  whom  I  examined 
many  years  ago,  that  a  chronic  paralysis  is  the  outcome  of 
a  diphtheritic  palsy  which  had  set  in  a  few  weeks  after  a 
severe  throat  affection. 

Bear  in  mind  the  possibility  of  cardiac  or  renal  compli- 
cations. The  paralysis  of  the  palate,  however  disagreeable 
it  may  be  to  the  child,  rarely  leads  to  serious  complications, 
but  if  the  muscles  of  the  pharynx  are  involved  there  is 
danger  of  food  entering  the  respiratory  tract,  and  of  a  sub- 
sequent pneumonia.  A  child  seen  recently  in  consultation 
was  in  danger  of  death  from  this  cause. 

Other  things  being  equal  the  earlier  the  paralysis  appears  after  the  initial 
disease,  and  the  more  quickly  it  reaches  an  extreme  development,  the  more 
serious  the  case  and  the  more  imminent  the  danger  to  life.  It  is  difficult  to 
forecast  the  exact  length  of  time  which  will  elapse  before  complete  recovery 
sets  in,  but  it  is  safe  on  the  whole  to  be  guided  by  the  same  rules  as  in  the 
case  of  any  other  peripheral  nerve  palsy.  If  the  electrical  examination  shows 
an  entire  loss  of  response  to  the  faradic  current  with  an  altered  response  to 
the  galvanic  current,  many  weeks  will  in  all  probability  elapse  before  recov- 
ery sets  in.  If  there  are  no  marked  changes  in  the  electrical  conditions  of 
the  nerves  and  muscles  the  degeneration  or  the  inflammation  of  the  nerve  is 
very  slight  and  the  paralysis  will  be  recovered  from  more  quickly. 

Treatment.— The  majority  of  cases  of  diphtheritic 
palsy  will  get  well  without  any  treatment,  but  it  is  of  the 
utmost  importance  at  all  times  to  maintain  the  strength  of 
the  patient  at  par,  and  to  be  prepared  for  any  sudden  com- 
plications. If  the  palate  is  paralyzed  and  there  is  regur- 
gitation of  food  through  the  nose  it  will  be  better  to  feed  the 
child  by  solids  than  by  liquids ;  if  in  consequence  of  paraly- 
sis of  the  pharynx  and  of  the  upper  air-passages  there  is 
danger  of  food  entering  the  respiratory  tract  it  may  be  best 
to  feed  the  child  for  a  time  per  rectum,  or  else  to  use  the 
feeding-tube  through  the  nose  or  the  mouth.  In  the  case 
of  children  weakened  by  the  diphtheritic  process  there  is 
danger  of  death  from  exhaustion,  and  no  time  should  be 
lost  in  employing  this  method  of  feeding  rather  than  to 
starve  the  child  while  hoping  for  a  spontaneous  recovery. 
On  the  other  hand,  even  while  the  rectal  feeding  is  kept  up 


246  THE   NERVOUS  DISEASES   OF   CHILDREN. 

efforts  should  be  made  every  now  and  then  to  have  the 
child  take  its  food  in  the  natural  way.  If  in  any  case  of 
diphtheritic  palsy  there  is  the  slightest  irregularity  or  weak- 
ness of  the  pulse,  cardiac  stimulants  should  be  given,  above 
all  digitalis  and  small  doses  of  strychnia.  If  respiratory 
weakness  threatens,  the  prompt  use  of  the  faradic  current, 
as  advised  by  Duchenne,  is  quite  in  order.  Direct  excita- 
tion of  the  phrenic  nerves  by  a  slowly  interrupted  faradic 
current  will  have  the  best  results.  The  electrical  currents 
should  be  employed,  according  to  the  principles  laid  down 
in  other  chapters,  for  the  treatment  of  paralyzed  parts. 

The  usual  tonics — iron,  quinine,  arsenic,  digitalis,  and 
strychnia — should  be  given,  but  no  one  would  venture  to 
assert  that  any  one  of  these  has  any  special  curative  action 
except  in  the  way  of  keeping  up  the  general  condition. 
Indeed  there  is  no  better  method  of  treating  diphtheritic 
palsy  than  by  carefully  administering  food,  and  making 
sure  that  everything  taken  into  the  system  will  help  to 
improve  the  nutrition  of  the  child.* 


LEAD    PARALYSIS. 

A  few  pages  are  to  be  devoted  to  this  special  form  of  paralysis,  in  order  to 
emphasize  the  fact,  which  was  proved  conclusively  by  Dr.  Putnam,  that  chil- 
dren are  no  less  susceptible  than  adults  to  the  effects  of  lead-poisoning.  Dr. 
Putnam  has  not  only  shown  that  entire  families  have  been  poisoned  by  lead, 
but  in  a  series  of  examinations,  made  for  the  purpose  of  determining  the 
presence  of  lead  in  the  urine  of  persons  who  were  supposed  to  be  healthy,  he 
found  considerable  quantities  in  the  urine  of  father,  mother,  and  two  of  the 
children  of  one  family,  all  of  them  being  in  fair  health  ;  a  third  child,  how- 
ever, for  some  unknown  reason,  did  not  exhibit  any  traces  of  the  poison. 
The  water  drank  by  this  family  ran  for  some  distance  through  lead  pipes 
and  was  found  to  contain  a  large  quantity  of  lead.  If  present  in  small  quan- 
tities, lead  is  practically  harmless.  Knowing  the  varied  susceptibility  of 
different  persons  to  lead,  and  having  proved  its  presence  in  the  urine  of  per- 
sons who  exhibited  no  symptoms  of  lead-poisoning,  Dr.  Putnam  is  right  in 
urging  that  the  possibility  of  lead-poisoning  should  be  taken  into  considera- 
tion in  all  obscure  cases. 

As  for  the  symptoms  of  lead-poisoning  in  children,  they  differ  but  little 
from  those  presented  by  adults,  and  are  in  entire  keeping  with  the  symptoms 

*  Since  the  above  was  written,  Behring's  important  discoveries  have  been  made 
known.  Whether  diphtheritic  palsy  can  be  affected  by  injections  of  the  serum  remains 
to  be  seen  ;  but  it  is  my  impression  that  paralytic  symptoms  have  become  less  frequent. 
Oppenheim  does  not  share  this  view. 


MULTIPLE    NEURITIS.  247 

of  multiple  neuritis  due  to  other  causes.  The  paralysis  is  most  frequently 
developed  in  the  extensor  groups  of  the  forearm,  resulting  in  characteristic 
drop-wrist  (Fig.  56).  The  muscles  affected  are  supplied  chiefly  by  the  mus- 
culo-spiral  nerve  ;  the  supinator  longus,  and  the  extensor  of  the  thumb  usu- 
ally escaping.  This  escape  of  the  supinator  longus  is  frequently  of  service  in 
helping  to  distinguish  the  disease  from  a  simple  traumatic  musculo-spiral 
neuritis,  for  in  the  latter  case  all  the  muscles  supplied  by  this  nerve  are  about 
equally  affected,  whereas  lead,  like  other  toxic  agents,  such  as  alcohol  and 
arsenic,  shows  a  selective  preference  for  groups  of  muscles  that  are  function- 
ally, not  structurally,  related.  In  adults  it  is  rare  to  find  other  muscles  seri- 
ously affected,  except  those  of  the  upper  extremities,  although  if  careful 
examination  is  made  of  such  patients,  a  slight  weakness  in  the  lower  extrem- 
ities and  a  diminution  of  the  reflexes  will  be  found  to  be  present.  I  have 
myself  seen  two  cases  of  lead  palsy  in  the  adult  in  which  the  entire  affection 
was  restricted  to  the  lower  extremities.  In  one  case  the  adductor  muscles  of 
the  thighs  were  the  only  ones  affected.  In  children  the  involvement  of  the 
lower  extremities  is  not  exceptional,  and  when  it  does  occur,  is  just  as  apt  as 
not  to  occur  earliest  in  the  course  of  the  disease,  the  upper  extremities  being 
affected  after  the  lower  extremities  have  been  paretic  or  paralyzed. 

While  the  symptoms  are  practically  identical  with  those  of  the  adult,  the 
diagnosis  of  lead  paralysis  will  be  all  the  more  readily  impressed  upon  the 
physician  by  the  occurrence  of  other  symptoms.  Thus  the  child  is  apt  to  ex- 
hibit a  general  cachectic  appearance  ;  also  a  peculiar  ashen  hue  of  the  skin, 
severe  headaches,  and  marked  disturbances  of  digestion.  Soltmann  expressed 
the  opinion  some  years  ago  that  lead  colic  was  rare  in  children.  It  is  more 
correct  to  assert  that  it  is  difficult  to  distinguish  lead  colic  from  other  forms 
of  colic  so  frequent  in  children.  Disturbance  of  digestion  due  to  constipation 
is  the  rule,  but  constipation  may  alternate  with  diarrhoea  and  with  severe 
attacks  of  colic  ;  the  colic  being  distributed  over  the  entire  abdomen  and  not 
in  the  vicinity  of  the  umbilicus,  as  is  generally  the  case  in  the  adult.  Vomit- 
ing frequently  occurs,  and  the  vomitus  may  be  yellowish  in  color.  The  ab- 
domen may  be  retracted,  but  in  many  of  the  cases  one  or  all  of  these  symp- 
toms may  be  absent. 

If  the  lead  poison  attacks  the  brain,  the  tendency  to  convulsions  will  be 
very  pronounced,  and  convulsions  may  possibly  be  the  cause  of  death.  In  the 
cases  reported  by  Karsch  and  Stewart  (quoted  by  Putnam),  the  children  died 
in  convulsions,  one  of  them  with  a  mild  form  of  delirium.  Gowers  supposes 
that  epileptic  attacks  may  occasionally  originate  in  lead-poisoning,  but  Put- 
nam, whose  experience  in  this  matter  is  greater  than  that  of  any  other  author, 
is  inclined  to  think  that  such  a  sequence  of  events  has  not  been  firmly  proven. 

The  lead  line,  which  is  of  such  assistance  to  us  in  diagnosticating  lead- 
poisoning  in  the  adult,  is  not  so  frequently  observed  in  children,  although  in 
one  epidemic  forty-two  cases  of  the  sort  were  reported.  The  better  condition 
of  the  teeth  in  children  is  unquestionably  the  explanation  of  this. 

The  pathology  and  pathological  anatomy  of  lead-poisoning  are  still  sub- 
jects for  discussion.  That  lead  acts  most  powerfully  on  the  peripheral  nerves 
cannot  be  doubted,  and  that  in  the  majority  of  cases  lead  paralysis  is  the  ex- 


248  THE  NERVOUS  DISEASES   OF  CHILDREN. 

pression  of  a  neuritis  it  is  also  safe  to  assume.  Gombault  insists  that  lead 
causes  a  periaxillary  neuritis,  the  sheath  of  the  nerve  being  more  affected 
than  the  axis-cylinder  and  relatively  healthy  portions  of  nerve  intervening 
between  diseased  parts.  These  changes  were  found  in  guinea-pigs  that 
were  not  paralyzed,  and  Putnam  suggests  very  correctly  that  such  changes  in 
the  myelin  without  paralysis  may  account  for  the  fact  that  in  man  the  elec- 
trical reactions  are  very  often  altered  before  any  actual  paralysis  appears. 
Harnack  and  others  have  suggested  that  lead  has  a  direct  action  upon  the 
muscles  rather  than  upon  the  nerves,  but  the  experimental  researches  of 
Stieglitz,  published  a  few  years  ago,  have  brought  the  entire  discussion  to  a 
temporary  and  satisfactory  close  by  showing  that  lead  produces  changes  in 
the  spinal  cord.  Onuf,  v.  Monakow,  and  others  have  reported  upon  lead 
poliomyelitis. 

Prognosis. — The  prognosis  of  lead-poisoning  is,  as  a  rule,  entirely  favor- 
able. There  is  more  danger  from  the  general  nutritional  disturbances  and 
from  the  effect  of  the  poison  upon  the  brain,  than  from  the  lesion  of  the 
peripheral  nerves  and  of  the  spinal  cord,  which  may  have  given  rise  to  the 
paralysis.  If  the  cachectic  condition  is  recovered  from  it  is  more  than  likely 
that  the  patient  will  regain  considerable  power  in  the  legs  in  the  course  of  a 
month  or  two,  but  the  actual  time  that  elapses  before  complete  recovery  sets 
in  may  vary  as  much  in  lead  palsy  as  it  does  in  other  forms  of  multiple 
neuritis,  and  it  need  not  be  a  matter  of  surprise  if  a  child  poisoned  by  lead 
shows  the  effects  of  such  poisoning  for  six  months,  or  even  a  year  ;  but  under 
all  conditions  it  is  a  source  of  satisfaction  to  be  able  to  predict  complete  re- 
covery eventually. 

Treatment. — If  the  patient  is  in  the  acute  stage  of  lead-poisoning  the 
same  measures  should  be  employed  which  are  advised  in  other  cases  of  acute 
metallic  intoxication.  If  the  patient  is  in  a  subacute  or  chronic  stage,  an  at- 
tempt should  be  made  to  eliminate  the  poison  through  the  kidneys  and  skin. 
The  iodides  have  become  the  classical  remedy.  It  is  better  to  clear  the  sys- 
tem of  the  lead  gradually  than  to  attempt  to  eliminate  the  poison  in  a  very 
few  days.  Doses  of  from  five  to  ten  grains  of  the  iodides  three  times  a  day 
will  be  quite  sufficient  for  all  purposes,  and  these  should  be  administered  in 
some  alkaline  water  which  will  stimulate  the  kidneys  to  greater  activity. 
Warm  baths  should  be  given  every  day,  or  every  other  day,  in  order  to  help 
on  the  same  cause. 

After  everything  has  been  done  to  eliminate  the  lead,  the  attention  should 
be  directed  entirely  to  the  treatment  of  the  paralyzed  parts,  and  in  this  re- 
spect the  use  of  active  massage  and  electricity,  in  the  manner  so  often  re- 
ferred to,  is  advisable.  The  general  condition  of  the  patient  will  also  call 
for  the  active  exhibition  of  blood  tonics,  such  as  iron,  arsenic,  and  cod-liver 
oil. 


DISEASES   OF   THE   SPINAL   CORD. 


CHAPTER   XIV. 

INFANTILE    SPINAL    PARALYSIS  — THE    ESSENTIAL    PARAL- 
YSIS   OF    CHILDREN— POLIOMYELITIS   ANTERIOR   ACUTA. 

Few  diseases  present  as  many  interesting-  problems  as 
does  infantile  spinal  paralysis.  While  the  diagnosis  of  the 
disease  is  an  easy  one,  there  is  considerable  doubt  as  to  its 
true  etiology;  its  pathology,  which  was  supposed  to  be 
firmly  established,  is  still  under  discussion,  and  the  treat- 
ment of  the  disease  deserves  a  most  careful  consideration. 
A  full  history  of  infantile  spinal  paralysis  would  convey  a 
very  fair  idea  of  many  of  the  important  advances  made  in 
neurological  science  within  the  last  thirty  years.  In  no 
disease  can  the  improved  methods  of  studying  the  morbid 
anatomy  of  the  spinal  cord  be  put  to  better  use,  and  in  few 
diseases  can  the  finer  problems  of  electrical  changes,  of  the 
localization  of  spinal  functions,  be  studied  to  greater  ad- 
vantage than  in  this  acute  spinal  palsy. 

The  clinical  features  of  acute  anterior  poliomyelitis  were  recognized  many- 
years  ago  by  the  German  orthopedic  surgeon  Heine,  and  his  description  of 
the  disease  still  holds  good,  with  few  exceptions,  to  the  present  day.  If  we 
add  to  his  description  of  the  disease  what  has  been  discovered  since  his  day, 
regarding  the  electrical  reactions  and  the  behavior  of  the  reflexes,  we  shall 
have  an  entirely  satisfactory  account  of  all  the  symptoms  of  the  disease. 
But  since  the  days  of  Heine  innumerable  French,  German,  English,  and 
American  authors,  among  whom  we  may  name  Duchenne,  Charcot,  Marie, 
Erb,  Seguin,  Strumpell,  Seeligmuller,  Siemerling,  Sinkler,  Mary  P.-Jacobi, 
and  a  host  of  others,  have  contributed  to  our  knowledge  of  this  disease. 
More  recent  authors,  whose  elaborate  researches  have  added  greatly  to  the 
proper  conception  of  the  pathology  of  the  disease,  will  be  referred  to  later  on 
in  connection  with  this  special  subdivision  of  the  subject. 

Symptoms. — Acute  anterior,  poliomyelitis  comes  on,  in 
the  vast  majority  of  the  cases,  after  the  fashion  of  an  acute  in- 
fectious disease.     Its  onset  is  signalized  by  fever,  vomiting. 


250 


THE  NERVOUS  DISEASES   OF  CHILDREN. 


convulsions,  and  even  coma.  All  these  symptoms  may  last 
for  a  few  hours,  or  even  a  few  days,  after  which  time  they 
gradually  subside  and  give  way  to  the  paralysis,  which  is 

the  most  permanent 
feature  of  the  disease. 
The  paralysis  is  typi- 
cal of  all  the  palsies 
which  are  due  to  a  le- 
sion in  the  second  di- 
vision of  the  motor 
tract.*  This  is  equiv- 
alent to  saying  that 
the  paralysis  is  of  the 
flaccid  order;  that  it 
is  associated  with  at- 
rophy ;  that  the  elec- 
trical reactions  in  the 
paralyzed  parts  are  al- 
tered, and  that  the  re- 
flexes in  the  parts  af- 
fected are  diminished 
or  lost.  The  very  sud- 
denness of  the  onset  is 
extremely  character- 
istic of  the  disease. 
There  are,  as  a  rule, 
no  prodromata,  and 
even  in  cases  in  which 
such  prodromal  symp- 
toms have  been  re- 
ported, it  is  doubtful 
whether  their  occur- 
rence was  not  a  mere 
coincidence.  The  fe- 
ver varies  between 
102°  and  1040  F.,  rarely  exceeding  the  latter  for  any  consid- 
erable period  of  time.  The  fever  generally  lasts  for  twenty- 
four  or  forty-eight  hours,  though  in  some  cases  in  which 

*  This  includes  the  ganglion  cell  in  the  anterior  horns,  the  anterior  roots,  the  pe- 
ripheral nerve,  and  the  muscle. 


Fig.  58.— Case  of  Infantile  Spinal  Palsy;  Paralysis 
and  Atrophy  of  Left  Leg  chiefly. 


INFANTILE   SPINA  J.   PARALYSIS. 


25I 


all  the  symptoms  show  marked  severity  the  fever  may  last 
for  an  entire  week.  There  is  no  doubt,  however,  that  in  a 
few  cases  of  typical  spinal  infantile  paralysis  no  fever  what- 
ever occurs.  Seguin  was  inclined  to  doubt  this  point,  but 
the  general  consensus  of  opinion  is  in  favor  of  the  occur- 
rence of  such  an  apyretic  con- 
dition. The  vomiting  which  ac- 
companies the  fever,  and  sets 
in  at  a  very  early  period  of  the 
disease,  may  resemble  the  cere- 
bral type,  and  is  independent  of 
any  gastric  disturbance.  The 
convulsions  are  quite  common 
during  the  first  day  of  the  dis- 
ease, are  occasionally  repeated 
during  the  first  three  days,  some- 
times during  the  first  week. 
They  are  of  the  order  of  general 
convulsions,  and  in  this  respect 
can  be  distinguished  from  the 
convulsions  which  occur  during 
the  earlier  stage  of  acute  cere- 
bral diseases.  In  some  cases  of 
poliomyelitis  anterior  acuta  the 
convulsions  are  entirely  absent. 
Coma  is  on  the  whole  very  much 
rarer  than  convulsions,  and,  if 
present,  may  be  taken  to  indi- 
cate a  tolerably  severe  form  of 
the  disease.  The  coma  is  not  as 
profound,  and  not  of  as  long  duration  as  in  many  acute  cer- 
ebral diseases. 

I  have  drawn  this  analogy  between  the  acute  cerebral  and  the  acute 
spinal  disease,  because  as  a  matter  of  fact  a  sharp  differentiation  between 
the  two  is  often  impossible  in  the  earlier  days  of  the  disease ;  and  many  a 
case  of  incipient  acute  anterior  poliomyelitis  has  been  diagnosticated  by 
skilful  physicians  as  the  first  stage  of  a  meningitis,  of  cerebral  hemorrhage, 
and  what  not. 

If  these  symptoms  of  the  initial  stage  are  not  well  de- 
veloped it  stands  to  reason  that  but  few  physicians  would 


Fig.  59.—  Case  of  Acute  Infantile 
Cerebral  Palsy  for  Comparison 
with  Fig.  58. 


252  THE  NERVOUS  DISEASES   OF  CHILDREN. 

be  willing  to  make  the  diagnosis  of  a  spinal  infantile  palsy 
with  absolute  certainty.  The  diagnosis  becomes  positive 
only  after  the  recognition  of  the  form  of  palsy  present. 

The  paralysis  may  possibly  be  present  from  the  very 
start,  but  it  is  very  often  overlooked  in  the  presence  of  the 
other  symptoms  which  appear  to  be  so  much  more  serious. 
I  have  not  infrequently  diagnosticated  this  spinal  paralysis 
during  the  first  ten  hours  after  the  onset  of  the  first  symptoms. 
The  paralysis  is  widely  distributed  at  the  start.  It  may  in- 
volve all  the  four  extremities,  and  may  even  involve  parts 
supplied  by  the  lower  cranial  nerves  (bulbar  paralysis)  as 
observed  by  Medin.  In  some  few  cases  disturbances  of 
speech  (dysarthria  rather  than  aphasia)  have  been  noticed. 
But  the  initial  wide-spread  paralysis  rapidly  diminishes 
within  a  few  days,  or  within  the  first  week,  and  before  long 
the  physician  will  be  able  to  recognize  the  parts  which 
will  remain  permanently  paralyzed,  or  at  least  to  recognize 
those  parts  which  will  remain  permanently  more  affected 
than  others;  for  it  is  not  until  after  the  lapse  of  some  con- 
siderable time,  say  from  two  to  four  weeks,  that  the  retro- 
gression of  the  paralysis  ceases  and  an  inference  can  be 
drawn  as  to  the  parts  that  will  be  permanently  maimed. 
This  retrogression  of  the  paralysis  has  been  insisted  on  by 
many  authors  as  the  most  important  feature  of  the  early 
stages  of  the  disease,  but  it  should  be  added  that  in  some 
instances  the  paralysis  is  seen  to  increase  for  a  few  days 
after  the  initial  stormy  symptoms  have  passed,  then 
reaches  its  climax,  and  from  this  time  on  begins  to  recede 
until  the  few  groups  of  muscles  that  are  more  seriously  dis- 
eased give  an  idea  of  the  amount  of  permanent  injury  done. 

The  permanent  paralysis  may  affect  one  or  more  parts  of  the  body,  but 
the  lower  extremities  are  much  more  frequently  affected  than  the  upper.  In 
her  article  in  Pepper's  "  System  of  Medicine,"  Mary  Putnam-Jacobi,  quoting 
from  Duchenne  and  Seeligmuller,  shows  that  the  paralysis  was  most  fre- 
quently distributed  as  follows  :  Left  lower  extremity  in  34  cases  ;  right  lower 
extremity,  40  times ;  right  upper  extremity  and  left  upper  extremity,  23 
times ;  all  four  extremities,  7  times ;  both  upper  extremities,  3  times ;  both 
lower  extremities,  23  times  ;  left  upper  and  lower  extremity,  twice.;  right  up- 
per and  lower  extremity,  once  ;  right  upper  and  left  lower  extremity,  3  times  ; 
muscles  of  trunk  and  abdomen,  once.  Of  these  137  cases  it  will  be  seen  that 
the  vast  majority  involve  the  lower  extremities.      The  paraplegic  and  mono- 


INF  A  NTH,  J:    SPINAL    PARALYSIS. 


253 


plegic  form  are  by  far  the  most  frequent,  while  among  monoplegias  an  affec- 
tion of  the  lower  extremity  is  more  frequent  than  that  of  the  upper ;  paralysis 
of  an  upper  extremity  is,  however,  not  so  rare  but  that  it  is  seen  often 
enough  in  every  clinic.  Special  mention  should  be  made  of  a  paralysis  of 
the  deep  muscles  of  the  back  (which  are  involved  in  a  number  of  the  cases) 
which  gives  rise  to  extreme 
lateral  curvature  of  the  spine. 
The  hemiplegic  variety  is  very 
rare  indeed. 

The  further  study  of  the 
exact  distribution  of  the  pa- 
ralysis in  poliomyelitis  has 
brought  out  a  number  of  in- 
teresting facts.  E.  Remak  in 
particular  has  shown  that  the 
parts  paralyzed  were  function- 
ally, not  anatomically,  related. 
In  the  upper  extremity  the  im- 
munity of  the  supinator  longus 
in  spite  of  paralysis  of  all  of  the 
extensor  muscles  in  the  fore- 
arm brings  out  this  peculiarity 
as  well  as  its  affection  in  con- 
nection with  paralysis  of  the 
deltoid,  the  biceps,  and  the 
brachialis  anticus.  This  latter 
form  of  paralysis  corresponds 
with  the  upper-arm  type.  In 
the  lower  extremity  the  pero- 
neal group  of  muscles  is  more 
frequently  affected  than  any 
other ;  next  in  frequency  the 
posterior  tibial ;  then  the  an- 
terior thigh  muscles,  and  least 
frequently  of  all,  the  posterior 
thigh  muscles.  The  tibialis 
anticus  is  generally  paralyzed 
in  connection  with  the  quad- 
riceps extensor.  These  muscles  are  supplied  by  different  nerves,  but  are 
associated  in  the  extension  movement  of  the  leg  during  walking.  The  in- 
volvement of  associated  muscles  in  poliomyelitis  would  furnish  the  data  for 
the  study  of  spinal  localization  if  the  disease  led  more  frequently  to  a  fatal 
issue  and  to  post-mortem  examinations. 

An  entire  extremity,  or  a  large  group  of  muscles,  may  be  permanently 
paralyzed ;  in  some  cases  the  loss  of  function  may  be  restricted  to  a  single 
muscle.  There  is  no  little  difficulty  at  times  in  making  out  the  one  or  more 
muscles  which  have  been  permanently  affected,  and  in  a  number  of  cases 


Fig.  60. — Paralysis  of  Upper-arm,  with  Atrophy 
(Left  Side)  due  to  an  Attack  of  Poliomyelitis 
in  Early  Childhood. 


254  THE  NERVOUS  DISEASES   OF   CHILDREN. 

which  have  come  under  my  observation  a  difference  in  the  electrical  behavior 
has  been  the  only  safe  way  of  determining  which  muscle  showed  a  departure 
from  the  normal. 


In  addition  to  this  retrogressive  form  of  paralysis,  which 
may  at  times  be  widely  distributed  and  at  other  times  curi- 
ously limited,  we  have  to  notice  the  rapidly  developing 
atrophy  of  the  paralyzed  muscles.  I  have  seen  a  marked 
difference  between  a  paralyzed  leg  and  the  other  normal 
member  within  three  days  of  the  first  onset  of  the  disease, 
and  it  is  not  at  all  unusual  to  recognize  the  wasting  of  the 
limb  by  superficial  inspection  within  the  first  week  or  two. 
The  wasting  is  developed  entirely  in  keeping  with  the  dis- 
tribution of  the  paralysis,  and  this  is  so  true  that  in  one  in- 
stance, which  I  can  recall,  the  atrophy  as  well  as  the  paral- 
ysis was  evidently  restricted  to  the  upper  portion  of  the 
tibialis  anticus.  In  the  case  of  the  deltoid  and  the  trapezius 
the  clavicular  portions  only  may  be  affected.  The  majority 
of  the  paralyzed  limbs  present  a  generally  slender  appear- 
ance. In  some  instances  the  difference  between  the  two 
limbs  may  not  amount  to  more  than  an  inch  or  two  in  cir- 
cumference, in  others  the  difference  may  be  extreme,  and  it 
is  not  unusual  to  find  a  paralyzed  extremity  that  is  scarcely 
half  the  size  of  the  normal  one.  The  wasted  paralyzed  part 
makes  the  impression  of  an  entirely  limp,  useless  append- 
age, that  is  at  the  mercy  of  the  parts  whose  muscles  are 
still  in  a  tolerable  state  of  preservation.  In  addition  to  the 
reduction  of  the  muscles  the  subcutaneous  tissue  and  the 
fat  often  disappear,  whereas  in  a  few  cases  of  children  that 
were  previously  well  nourished,  the  muscular  wasting  may, 
for  a  time,  be  concealed  by  the  very  considerable  presence 
of  adipose  tissue.  In  addition  to  the  reduction  in  circum- 
ference the  atrophied  parts  do  not  grow  as  the  normal  parts 
do,  whence  it  follows  that  short  limbs  are  not  infrequently 
the  result  of  an  early  attack  of  poliomyelitis. 

The  diseased  member  presents  a  few  other  symptoms 
which  render  the  paralysis  easy  of  detection.  The  skin 
over  the  paralyzed  muscles  often  has  a  slightly  shrivelled 
appearance ;  more  often  still  it  is  blue,  cold,  and  clammy,  so 
that  by  the  mere  touch  of  the  two  lower  extremities  the 


INFANTILE   SPINAL   PARALYSIS.  255 

physician  can  recognize  the  one  that  is  paralyzed.  This 
difference  in  the  temperature  of  the  parts  is  probably  due  to 
the  improper  blood-supply  and  to  the  natural  shrinkage  of 
blood-vessels  that  have  no  duties  to  perform,  or  eLse  send  a 
lessened  supply  to  diminutive  muscles. 

The  behavior  of  the  electrical  reactions  is  of  the  utmost 
importance.  With  rare  exceptions  the  paralyzed  muscles 
and  the  nerves  supplying  them  exhibit  a  complete  reaction 
of  degeneration.  Both  the  nerves  and  the  muscles  fail  to 
respond  to  the  faradic  current ;  the  nerves  cannot  be  ex- 
cited by  ordinary  galvanic  stimulation,  and  the  muscle  re- 
sponds in  sluggish  fashion,  and  often  with  an  altered  gal- 
vanic formula,  the  anodal  closure  contraction  being  equal 
to,  or  greater  than,  the  cathodal  closure  contraction  (see 
pp.  44  and  45).  These  changes  in  electrical  behavior  come 
on  very  early  after  the  onset  of  the  disease.  From  several 
examinations  which  I  have  been  able  to  make  in  very  early 
stages  of  the  disease,  I  can  assert  that  both  the  faradic  and 
the  galvanic  response  of  the  parts  paralyzed  are  increased 
for  the  first  two  days,  but  then  become  rapidly  diminished  ; 
the  nerves  and  muscles  soon  fail  to  respond  to  the  faradic 
current,  while  the  galvanic  response  may  remain  increased 
for  a  very  long  period  of  time ;  this  grows  more  sluggish, 
and  finally  there  is  no  contraction  except  to  very  strong 
currents.  It  can  be  stated  with  some  degree  of  certainty 
that  those  parts  which  continue  to  respond  well  to  faradism, 
say  after  the  lapse  of  a  week  or  more,  will  not  remain  per- 
manently  paralyzed,  whereas  the  utter  loss  of  function  for  a 
varying  period  of  time  of  those  muscles  which  at  once 
fail  to  respond  to  the  faradic  current  may  be  safely  pre- 
dicted. During  the  later  stages  of  the  disease  the  return 
of  the  faradic  response  in  any  muscle,  or  a  normal  behavior 
during  galvanic  stimulation,  will  lead  us  to  infer  that  the 
muscle,  or  muscles,  in  question  mav  recover  their  previous 
function,  at  least  to  a  limited  extent ;  but  muscles  which 
exhibit  marked  electrical  changes  for  a  considerable  period 
of  time  have  suffered  a  very  serious  injury. 

The  reflexes  are  diminished  in  cases  of  poliomyelitis 
anterior ;  but  this  is  true  only  of  those  reflexes  which  are 
associated  with  the  normal  function  of  the  paralyzed  parts. 


256  THE  NERVOUS  DISEASES   OF   CHILDREN. 

Thus  in  a  large  proportion  of  the  cases  of  poliomyelitis 
anterior  the  knee-jerk  is  absent ;  but  it  is  needless  to  say 
that  it  is  not  absent  in  cases  of  the  cervical  type,  nor  even 
in  those  cases  in  which  only  the  posterior  tibial  muscles  or 
the  muscles  of  the  feet  are  involved. 

I  have  in  a  number  of  instances  been  confronted  by  other  physicians  with 
an  expression  of  grave  doubt  as  to  the  diagnosis  of  poliomyelitis  because  of 
the  presence  of  the  knee-jerk,  when  a  careful  examination  of  the  parts  paralyzed 
proved  that  the  paralysis  was  limited  to  the  extensor  muscles  below  the  knee 
and  to  the  posterior  groups  of  muscles,  while  the  quadriceps  extensor  and  all 
the  anterior  thigh  muscles  were  entirely  normal.  The  mere  presence  of  the 
knee-jerk  under  such  conditions  need  not,  therefore,  militate  against  the  cor- 
rectness of  the  diagnosis. 

If  all  the  muscles  of  a  given  part  are  equally  paralyzed 
and  equally  atrophied  no  contractures  will  set  in,  but  all  the 
parts  will  be  equally  limp.  Thus  the  leg  can  be  pushed  to 
and  fro  by  the  slightest  touch  (Punchinello  leg).  Since 
locomotion  and  station  are  the  results  of  an  accurate  bal- 
ancing between  the  extensor  and  flexor  groups  of  muscles, 
it  is  but  natural  to  expect  that  if  one  set  of  muscles  is  para- 
lyzed, the  opposing  muscles,  being  tolerably  normal  and 
having  less  work  to  do,  will  overact,  and  permanent  over- 
action  is  expressed  by  contracture.  As  a  rule  these  con- 
tractures do  not  appear  until  an  attempt  is  made  to  use  the 
affected  limbs,  but  in  a  few  instances — and  some  such  have 
come  under  my  notice — the  contractures  form  while  the 
child  lies  helpless  on  its  back  without  any  attempt  what- 
ever at  walking  or  at  using  the  limb.  It  would  seem  from 
this  that  some  more  active  cause  must  be  at  work,  and  it  is 
probable  that  the  inflammation  which  destroys  the  function 
of  some  cells  may  cause  an  irritation  of  neighboring  cells 
and  fibres,  and  thus  produce  contracture,  very  much  as  it 
it  is  produced  in  other  diseases  in  which  a  lesion  in  the  ad- 
jacent white  matter  acts  as  an  irritant  upon  the  ganglion 
cells  of  the  anterior  horns.  According  to  Volkmann  and 
•others  the  mere  weight  of  the  body  or  of  a  limb  may  cause 
a  deformity  if  the  muscles  surrounding  a  joint  are  paralyzed. 

The  most  frequent  deformities  are  as  follows  :  Pes  equinus,  or  equino- 
varus ;  pes  valgus  ;  genu  recurvatum  and  incurvatum ;  permanent  flexion 
(rare)  ;  all  these  deformities  are  evidently  promoted  by  the  attempt  to  use  the 


INFANTILE  SPINAL   PARALYSIS. 


257 


maimed  legs.  Deformity  of  the  hip  is  very  much  rarer  than  those  affecting 
the  knee  or  the  foot.  If  the  disease  involve  the  upper  extremities  extension 
of  the  wrist  is  common  in  the  presence  of  paralysis  of  the  flexors  ;  clawed 
hand  if  the  interossei  are  involved,  and  a  flexion  of  the  fingers  or  wrist  if  the 
extensors  chiefly  are  paralyzed.  The  elbow-joint  is  rarely  deformed,  but  is 
often  entirely  useless  if  the  upper-arm  muscles  are  paralyzed.  At  the  shoul- 
der-joint atrophy  of  the  deltoid  may  be  the  direct  cause  of  a  subluxation. 
The  most  serious  deformities  that  occur  are  those  due  to  a  paralysis  of  the 
abdominal  and  deep  spinal  muscles.  According  to  the  extent  and  number  of 
the  muscles  involved  we  may  have  a  scoliosis,  occasionally  a  kyphosis ;  but 
most  frequently  very  marked  lateral  curvature,  particularly,  in  the  cervical  re- 
gion, or  a  very  marked  lordosis  in  the  lumbar  region.  From  paralysis  of  the 
abdominal  muscles  the  abdomen  may  become  peculiarly  distorted,  and  in  one 
case  of  a  child  of  about  three,  which  I  saw  in  private  practice,  a  marked  lordosis 
of  the  lumbar  region  was  associated  with  what  appeared  to  be  an  enormous 
bulging  of  the  anterior  left  half  of  the  abdomen.  In  accounting  for  these  de- 
formities we  must  remember  that  in  addition  to  the  paralysis  of  the  muscles, 
the  arrested  growth  of  the  bones,  and  the  disturbance  in  the  general  develop- 
ment of  the  child  play  a  very  considerable  part. 

Before  leaving  the  symptomatology  of  the  disease  it  is 
important  to  insist  on  a  few  negative  symptoms  which  help 
to  corroborate  the  diagnosis  in  doubtful  cases.  It  is  gener- 
ally asserted  that  pain  is  entirely  absent.  This  is  true 
of  the  vast  majority  of  cases,  and  particularly  of  the  later 
stages  of  the  disease  ;  but  in  one  of  the  few  cases  which  I 
was  fortunate  enough  to  see  at  an  early  day,  pain  was  an  ex- 
tremely prominent  symptom.  In  such  cases,  therefore,  the 
absence  or  presence  of  pain  could  not  serve  as  a  sufficient 
factor  in  differentiating  the  disease  from  a  peripheral  neu- 
ritis, or  from  articular  rheumatism.  The  bladder  and  the 
rectum  are,  as  a  rule,  not  involved.  This  is  a  point  of  some 
importance  in  helping  to  differentiate  between  inflammation 
of  the  anterior  horns  and  a  general  myelitis — a  point  of  dif- 
ferential diagnosis  that  comes  up  frequently  enough  for  dis- 
cussion. The  entire  absence  of  cerebral  symptoms  after 
those  of  the  initial  stage  have  passed  away  will  help  to  dis- 
tinguish the  spinal  infantile  paralysis  from  many  cases  of 
cerebral  palsy. 

Summarizing  all  these  symptoms  we  may  state  that  the 
diagnosis  of  poliomyelitis  anterior  may  be  made  if  the 
paralysis,  however  widely  distributed,  or  however  narrowly 
limited,  and  in  whatever  part  of  the  body,  comes  on  after 


258  THE  NERVOUS  DISEASES    OF   CHILDREN. 

an  acute  onset  marked  by  fever,  vomiting,  and  convulsions, 
and  if  this  paralysis  is  associated  at  an  early  day  with 
atrophy,  with  changes  in  electrical  reactions,  and  with  a  loss 
of  reflex  activity  in  the  paralyzed  parts. 

Morbid  Anatomy  and  Pathology.  —  "  Poliomyelitis 
anterior  due  to  atrophy  of  the  ganglion  cells  of  the  anterior 
horns,"  has  become  a  byword  of  neurological  science.  It 
would  be  fortunate  indeed  if  such  a  simple  statement  as  this 
would  settle  the  much-disputed  question  of  the  morbid 
changes  underlying  the  disease. 

As  far  back  as  1863,  Cornil  published  a  case  of  a  woman  who  had  ac- 
quired a  sudden  paralysis  of  both  legs  at  the  age  of  two  years,  and  who  had 
died  of  carcinoma  at  the  age  of  forty-nine.  Cornil  found  the  spinal  cord 
much  smaller  than  normal,  and  thought  this  due  to  an  atrophy  of  the  anterior 
roots  and  of  the  an tero- lateral  white  matter.  He  referred  to  atrophic  gan- 
glion cells,  but  did  not  make  them  responsible  for  the  disease.  In  1865  Pre- 
vost  and  Vulpian  examined  another  case,  also  in  an  elderly  person,  who  had 
died  of  cerebro-spinal  meningitis.  She  had  had  a  paralytic  club-foot  with 
complete  atrophy  of  the  posterior  leg  muscles,  which  was  found  to  be  due  to 
an  atrophy  of  the  anterior  horns  of  the  left  side,  in  the  lumbar  enlargement. 
The  ganglion  cells  were  found  to  be  fewer  in  number,  and  the  antero-lateral 
white  matter  was  also  atrophied  on  the  same  side.  Very  much  the  same 
result  was  obtained  in  another  case  by  Clarke,  but  the  importance  of  disease 
of  the  gray  matter  and  of  the  relations  of  the  ganglion  cells  of  the  anterior 
horns  to  the  paralyzed  parts  was  not  fully  established  until  the  famous  publi- 
cation of  Charcot  and  Joffroy  in  1870,  who  made  the  atrophy  of  the  ganglion 
cells  entirely  responsible  for  all  the  symptoms  of  the  disease.  Their  studies 
were  based  also  upon  the  examination  of  the  spinal  cord  of  a  woman,  who 
died  at  the  age  of  forty,  but  who  had  acquired  an  infantile  paralysis  at  the 
age  of  seven  years.  The  ganglion  cells  in  the  lumbar  segment  were  dimin- 
ished, and  were  in  part  entirely  absent.  A  dense  sclerotic  tissue  marked  the 
disappearance  of  these  ganglion  cells. 

Since  the  publication  of  Charcot  and  Joffroy's  article,  it 
was  current  belief  that  the  chief  anatomical  changes  in  polio- 
myelitis were  those  involving  the  gray  matter  and  its  gan- 
glion cells;  that  the  morbid  process  might  extend  through- 
out the  entire  length  of  the  cord,  or  it  might  be  developed 
chiefly  in  the  cervical  or  lumbar  enlargement.  The  question 
arose  whether  the  inflammation  of  the  anterior  horns  is  pa- 
renchymatous in  character,  that  is,  a  primary  inflammation  of 
the  ganglion  cells,  or  whether  it  is  of  the  order  of  interstitial 
inflammations,  in  which  the  neuroglia  is  the  part  first  affected 


INFANTILE   SPINAL   PARALYSIS.  259 

and  the  ganglion  cells  are  destroyed  later  on.  This  dispute 
has  been  carried  on  to  the  present  day.  It  was  begun  by 
Roger  and  Damaschino,  and  has  been  continued  by  Leyden, 
Schultze,  Eisenlohr,  Bramwell,  Marie,  Marinescoand  others! 
Leyden  recognized  that  a  number  of  different  pathological 
processes  might  give  rise  to  the  clinical  symptoms  of  an 
infantile  spinal  palsy.  He  was  inclined  to  adopt  Charcot's 
theory  of  a  parenchymatous  inflammation,  but  in  one  case 
which  he  examined  he  thought  the  lesions  somewhat  similar 
to  those  described  by  Roger  and  Damaschino.  It  was  evi- 
dent that  this  question  could  not  be  satisfactorily  settled  if 
the  microscopical  investigations  were  restricted  to  cases  in 
which  many  years  had  passed  between  the  time  of  onset  of 
the  disease  and  the  death  of  the  patient. 

The  effort  has  been  made  to  study  recent  cases  of  the 
disease.     The   first  good   opportunity  was  presented  by  a 
very  extensive    epidemic   of   poliomyelitis   anterior   acuta 
observed  by  Professor  Medin,  of  Stockholm,  and  carefully 
described   by   his  pupil  John    Rissler,   who    recorded    the 
autopsies  on  three  cases.     Goldscheider,  Marie,  Redlich, 
and  Siemerling  have  also  published   the   results  of   post- 
mortem  examinations  of  children   who   died   very  shortly 
after  the  onset  of  the  disease.    French  and  German  authors 
have  helped  greatly  to  develop  a  fuller  knowledge  of  the 
anatomical  process,  and  their  results  are  in  direct  contra- 
diction of  the  opinions  of  Charcot  and  Joffroy,  which  have 
obtained  such  general  credence  in  neurology.     Nearly  all 
recent  writers*  have  come  to  the  conclusion  that  the  entire 
gray  matter  is  the  seat  of  interstitial  inflammation,  and  that 
the  changes  in  the  ganglion  cells  are  secondary.     Many  of 
the  authors  are  also  inclined  to  regard  poliomyelitis  as  an 
acute  infectious  disease,  in  spite  of  the  entire  absence,  up 
to  the  present  day,  of  proof  of  the  microbic  origin  of  the 
disease.      They   are   of   the   opinion    that    the  entire  gray 
substance  is  easily  affected  and  infected  by  the  poison,  and 
that  that  part  of  the  spinal  cord   is   most  easily  involved 
which  has  the  most  abundant  blood-supply. 

Goldscheider's  investigations,  which  were  most  carefully 
made,  point  to  the  important  role  played  by  the  blood-ves- 

*  Rissler  and  v.  Kahlden  upheld  Charcot's  views  for  many  years. 


26o 


THE   NERVOUS  DISEASES   OE   CHILDREN. 


sels  of  the  spinal  cord.  The  author  concludes  that  a  condi- 
tion of  irritation  is  present  in  the  walls  of  the  blood-vessels 
which  leads  to  a  dilatation  of  these  vessels  and  to  a  prolif- 
eration of  their  endothelial  elements.  From  this  the  morbid 
process  extends  to  the  neuroglia  and  produces  a  prolifera- 
tion of  its  cells.     The  changes  in  the  ganglion  cells  are  of  a 


'$1$$§£ 


m    |  /#7 


Fig.  6i. — Poliomyelitis  Anterior  ;  Part  of  an  Acute  Myelitis.  Death  of  child  (age,  two 
and  a  half  years)  eight  days  after  onset  of  complete  palsy  of  legs  and  arms.  (Sie- 
merling.)  Section  through  lumbar  segment,  showing  disruption  of  anterior  gray 
matter  from  hemorrhage  into  it.  B,  marginal  blood-vessel ;  A,  branch  of  anterior 
spinal  artery. 


degenerative  nature,  and  the  changes  in  them,  as  well  as  in 
the  nerve-fibres,  are  secondary  and  due  to  disease  of  the 
blood-vessels.  Goldscheider  has  also  shown  that  these  de- 
generative changes  occur  most  distinctly  in  the  vicinity  of 


INFANTILE   SPINAL   PARALYSIS. 


26l 


altered  blood-vessels,    and  that  the  degenerated  ganglion 
cells  lie  in  vascular  areas. 

The  cases  reported  by  Siemerling,  while  corroborating 
the  views  of  Goldscheider  and  others,  have  a  still  deeper 
significance.  They  show  that  in  some  instances  a  poliomy- 
elitis anterior  is  merely  a  part  of  a  general  myelitis  of  both 
the  gray  and  the  white  matter.  The  frequent  restriction 
of  the  process  to  the  anterior  cornua  must  be  ascribed  alto- 
gether to  the  peculiarities  of  arterial  blood  supply.     It  is 


WM  k 


.'>: 


Fig.  62. — Poliomyelitis  Anterior  of  Old  Standing,  showing  Disappearance  of  Ganglion 
Cells  on  Right  Side,  and  Shrinkage  of  Right  Half  of  Cord.  Drawn  from  a  section 
through  lower  cervical  region. 

doubtful,  however,  whether  the  hemorrhage  into  the  an- 
terior horns  is  often  as  destructive  as  in  one  of  Siemerling's 
cases  (Fig.  61).  All  these  investigations  prove  that  in  in- 
fantile spinal  palsy  the  inflammatory  process  is  interstitial 
not  parenchymatous.  It  may  be  limited  to  a  few  segments 
of  the  cord,  or  it  may  involve  the  greater  part  of  the  cord, 
and  may  extend  to  the  medulla  and  pons. 

For  the  changes  that  occur  in  consequence  of  this  early 
inflammation,  and  for  the  appearance  of  the  cord  in  the  later 


262  THE   NERVOUS  DISEASES    OF    CHILDREN. 

years,  we  may  refer  safely  enough  to  the  famous  publica- 
tions of  Charcot  and  others. 

There  is,  first  of  all,  a  disappearance,  often  complete,  of 
the  larger  ganglion  cells  of  the  anterior  horns,  and  the  few 
that  are  visible  are  altered  in  appearance.  The  nucleus  has 
disappeared,  the  cell-body  is  shrunken,  and  the  cell  con- 
tours are  entirely  different  from  the  normal,  every  trace  of 
the  cell  processes  having  disappeared.  But  the  ganglion 
cells  are  not  the  only  parts  that  undergo  changes  ;  the  en- 
tire gray  matter  shrinks,  and,  as  can  be  seen  in  the  annexed 
cut,  the  entire  gray  matter  in  one-half  of  the  spinal  cord  is 

shrunken    in    size,    and 
*=22SBft^ji  the  white  matter  of  the 

same  side  is  also  very 
much    less    in    volume 
than  that  of  the  opposite 
I  'By      half.    In  consequence  of 

y       the    changes    going  on 
in  the  gray  matter,  the 
columns  of  Clarke  dis- 
appear,   together    with 
„     ,  ,. .   .  ^,  other     nervous    struct- 

FiG.  63. — Poliomyelitis  Anterior.     Chronic  stage  ; 

section  through  sixth  cervical  segment,  showing  ureS.         1  xiese      Changes 

diminution  of  anterior  gray  matter  and  of  en-  are   unquestionablv  SeC- 

tire  half  of  risrht  side.    (Drawn  from  a  specimen  -.                     ,,            , 

, .  -,,  r     ■  t  a      u   n    r,v     v  ondarv  to  the  changes 

kindly  furnished  me  by  Dr.  Collins.)  J  o 

in  the  anterior  horns, 
and  considering  the  intimate  relation  between  the  anterior 
horns  and  the  remaining  part  of  the  gray  matter,  as  has 
been  brought  to  light  by  recent  anatomical  investigations, 
we  can  readily  understand  why,  in  consequence  of  disease 
in  one  part  of  the  gray  matter  causing  destruction  of  nerve- 
cells,  the  nerve-fibres  which  owe  their  life  and  nutrition  to 
such  cells  disappear  as  well.  The  anterior  nerve-roots  are 
smaller  than  the  corresponding  roots  of  the  sound  side. 
These  changes  are  also,  in  all  probability,  secondary  to  the 
changes  in  the  ganglion  cells. 

So  much  for  the  changes  to  be  observed  in  the  spinal 
cord  itself.  The  atrophied  muscles  also  present  character- 
istic conditions.  The  fibres  are  very  much  diminished  in  size, 
many  of  them  have  disappeared  altogether,  and  the  place 


INFANTILE   SPINAL   PARALYSIS.  263 

once  occupied  by  the  normal  fibres  is  largely  filled  by  adi- 
pose tissue.  There  is  in  these  cases  no  such  nuclear  prolif- 
eration and  no  hypertrophy  of  fibres  such  as  are  found  in 
the  muscles  of  patients  suffering  from  various  forms  of 
muscular  dystrophy.  But  even  the  presence  of  a  few 
hypertrophied  fibres  would  not  be  unusual,  as  the  stage  of 
hypertrophy  seems  to  indicate  an  incipient  irritation  which 
precedes  the  condition  of  atrophy.  Marie  has  gone  to  some 
trouble  to  show  that  even  the  bones  in  cases  of  poliomye- 
litis undergo  trophic  changes.  The  bones  are  smaller  than 
those  of  the  corresponding  healthy  member  and  appear 
more  rounded  on  cross-section  than  the  healthy  bone  does. 
Theory  of  the  Disease. — That  poliomyelitis  repre- 
sents an  acute  inflammatory  condition  of  the  anterior  gray 
matter  of  the  spinal  cord  is  conceded  on  all  sides,  but  the 
question  arises  what  the  origin  of  such  inflammation  may 
be.  The  only  satisfactory  explanation  at  the  present  day  is 
to  suppose  that  the  inflammation  is  the  result  of  an  acute 
infection  which  happens  to  be  located  in  the  spinal  cord, 
just  as  other  acute  infectious  diseases  show  a  predilection 
for  other  sites  in  the  body.  The  microbic  origin  has  not 
yet  been  satisfactorily  demonstrated,  but  all  the  clinical 
facts  point  toward  this  view,  and  the  close  dependence  of 
the  myelitic  process  upon  the  distribution  of  the  blood-ves- 
sels lends  further  color  to  this  theory.  The  infectious  ori- 
gin of  poliomyelitis  is  also  rendered  highly  probable  by  the 
frequent  observation  of  the  epidemic  occurrence  of  infantile 
spinal  paralysis.  Such  epidemics  have  been  recorded  by 
Medin  and  Briegleb  in  Europe,  and  by  Colmer  (1843)  and 
Caverly  (1894)  in  this  country.  Zappert  speaks  of  a  well- 
nigh  epidemic  occurrence  of  the  disease  in  Vienna  in  1898. 

For  several  years  past  I  have  recorded  carefully  the  cases  of  poliomyelitis 
in  dispensary  and  private  practice,  and  have  noticed  that  at  least  75  per 
cent,  began  between  the  months  of  July  and  October.  Medin  observed  five 
cases  in  the  spring  of  one  year,  and  between  August  and  November  he  had 
examined  altogether  forty-four  cases  of  poliomyelitis  which  had  begun  during 
this  period.  Sinkler  states  that  of  270  cases  213,  or  78.8  per  cent.,  were  at- 
tacked in  the  hot  months  of  the  year,  from  May  to  September,  inclusive. 

Marie  supposes  that  an  infectious  embolism  or  thrombo- 
sis in  one  or  more  of  the  branches  of  the  anterior  spinal 


264  THE  NERVOUS  DISEASES   OF   CHILDREN. 

artery  may  be  the  direct  cause  of  the  attack  of  poliomye- 
litis. 

Marie  is  inclined  also  to  infer  the  infectious  nature  of  poliomyelitis  from 
the  close  resemblance  between  polioencephalitis  and  poliomyelitis,  and  quotes 
approvingly  the  two  cases  of  Moebius  occurring  in  one  family,  in  which  one 
child  was  attacked  with  the  form  of  acute  cerebral  palsy,  and  the  other  child 
with  an  acute  spinal  palsy.  But  surely  this  proof  of  the  infectious  theory  of 
acute  cerebral  palsy  is  extremely  slender,  and  Moebius's  cases  might  well  be 
due  to  a  coincidence  rather  than  to  an  infection,  which  is  supposed  to  have 
caused  a  cerebral  paralysis  in  the  one,  and  a  spinal  paralysis  in  the  other 
child.  Nor  can  two  cases  occurring  in  the  same  family  be  considered  evi- 
dence of  an  epidemic  character  of  the  disease.  A  few  years  ago  two  chil- 
dren, cousins,  were  brought  to  me,  who  had  developed  acute  spinal  palsy 
within  two  weeks  of  one  another.  The  one  child  has  remained  severely 
paralyzed  in  both  lower  extremities  up  to  the  present  day,  the  other  child 
escaped  with  a  slight  paralysis  of  the  anterior  tibial  group.  The  theory  of 
infection  would  be  a  very  simple  one  to  hold  in  such  cases,  but  on  closer 
examination  it  was  found  that  both  these  children  had  developed  the  symp- 
toms of  their  disease  shortly  after  exposure  to  an  extremely  cold  surf  bath. 
The  infectious  theory  of  poliomyelitis  is  a  very  plausible  one,  but  we  cannot 
disregard  other  possible  causes,  and  refrigeration,  as  in  the  two  cases  just 
cited,  may  in  some  instances  be  a  powerful  factor  in  the  development  of 
poliomyelitis.  It  seems  to  me  that  those  authors  make  a  mistake  who  insist 
on  a  single  origin  for  such  a  frequent  disease  as  poliomyelitis.  In  a  very 
recent  article  (Brain,  Autumn,  1904),  Batten  arrives  at  the  same  conclusion: 
"  Such  thrombosis  (of  the  anterior  spinal  artery  or  its  branches)  may  be  pro- 
duced by  many  and  various  forms  of  infection  and  the  disease  is  not  due  to 
a  special  specific  infection."  Schultze  reports  that  he  has  found  Weichsel- 
baum's  diplococcus  in  the  cerebro-spinal  fluid  obtained  by  lumbar  puncture  ; 
he  thinks  that  occasionally  poliomyelitis  anterior  may  be  a  disguised  form  of 
cerebro-spinal  meningitis.  Marinesco  is  of  the  opinion  that  the  entrance  of 
micro-organisms  into  the  anterior  spinal  artery  produces  poliomyelitis  an- 
terior. Great  caution  must  still  be  exercised  in  the  interpretation  of  bacteri- 
ologic  findings. 

Differential  Diagnosis. — Meningitis  can  be  excluded 
if  there  are  no  other  signs  of  a  meningeal  process,  except 
possibly  coma  and  convulsions.  In  cases  of  meningitis  these 
constitute  the  first  of  a  series  of  many  cerebral  symptoms, 
such  as  vomiting,  rigidity  of  the  neck,  headaches,  cranial 
nerve  affections,  and  the  like.  In  poliomyelitis  none  of  these 
symptoms  appear,  and  the  coma  and  convulsions  last  but  a 
relatively  short  time. 

There  is  little  difficulty  in  distinguishing  between  well- 


INFANTILE   SPINAL  PARALYSIS.  265 

developed  cases  of  acute  spinal  and  acute  cerebral  palsy  of 
children,  but  the  less  pronounced  types  of  these  diseases 
cannot  be  easily  distinguished  from  one  another  unless  a 
very  careful  examination  is  made  of  all  the  accompanying 
symptoms.  The  mode  of  onset  may  be  exactly  similar  in 
both  ;  it  is,  in  fact,  on  the  close  resemblance  between  the 
two  diseases  in  this  respect  that  Striimpell  was  led  to 
build  up  his  theory  of  the  analogy  between  the  two.  But 
aside  from  the  symptoms  of  onset,  the  clinical  features  are 
almost  diametrically  opposed  to  one  another.  The  follow- 
ing table  will  bring  out  these  symptoms  in  the  clearest  pos- 
sible manner: 

Acute  Spinal  Palsy.  Acute  Cerebral  Palsy. 

Onset  sudden,  with  fever,  coma,  and  Onset  sudden,  with  fever,  coma,  and 

convulsions.      Convulsions    rarely  convulsions.     Convulsions  apt  to  be 

repeated  after  first  few  days.  repeated. 

Paralysis     flaccid,     associated     with  Paralysis  spastic ;    no  atrophy  ;  asso- 

atrophy.  ciated   with   rigidity    and   contrac- 
tures. 

Paralysis  widely  distributed,  possibly  Paralysis  generally  hemiplegic,  some- 
involving  all    extremities,  or    nar-  times  diplegic  or  paraplegic.    Alono- 
rovvly  limited  to  one   member,  or  plegia  rare, 
even  a  single  group  of  muscles. 

Electrical  reactions  altered  (R.  D.).  Electrical  reaction  normal. 

Deep  reflexes  diminished  or  lost.  Deep  reflexes  exaggerated. 

Intellect  never  permanently  involved ;  Intellect  often  involved  ;  epilepsy  fre- 

no  epilepsy.  quent. 

Doubt  may  arise  as  to  the  differential  diagnosis  in  some 
cases  between  poliomyelitis  anterior  and  a  peripheral  (mul- 
tiple or  simple)  neuritis.  The  onset  may  be  equally  sudden 
in  both,  though  in  many  cases  of  neuritis  the  onset  is  much 
more  gradual  than  it  is  in  cases  of  poliomyelitis.  In  neu- 
ritis there  are,  as  a  rule,  fewer  symptoms  of  general  ner- 
vous disturbance  than  in  poliomyelitis  ;  but  in  those  forms 
of  neuritis  in  which  there  is  a  distinct  toxic  infection  the 
toxic  poisoning  may  produce  cerebral  symptoms  very 
closely  akin  to  those  met  with  in  the  earlier  stages  of  polio- 
myelitis. The  distribution  of  the  paralysis  may  be  the 
same  in  both  instances,  but  after  all  it  is  much  more  likely 
to  be  distributed  according  to  strict  anatomical  lines  in 


266  THE   NERVOUS  DISEASES    OF   CHILDREN. 

neuritis  than  in  poliomyelitis.  In  the  latter,  muscles  that 
have  a  common  function  are  very  apt  to  be  paralyzed  to- 
gether. In  former  days  the  presence  of  pain  along  nerve- 
trunks  and  along  nerve-branches  supplying  the  paralyzed 
muscles  was  supposed  to  be  a  safe  feature  of  differential 
diagnosis,  and  this  holds  good  in  a  majority  of  cases  ;  but 
according  to  my  own  experience  pain  may  be  present  in  the 
acute  stage  of  poliomyelitis,  and  if  the  child  is  too  young 
to  give  accurate  information  to  the  physician,  it  is  well- 
nigh  impossible  to  determine  whether  the  pain  is  a  gen- 
eral one  in  the  joints  or  whether  it  is  along  the  distribution 
of  the  peripheral  nerve-branches.  But  1  have  never  seen 
pain  persist  for  any  great  length  of  time  in  poliomyelitis, 
while  it  persists,  as  a  rule,  for  days  and  weeks  in  cases  of 
neuritis.  The  atrophy,  the  electrical  reactions,  and  the  re- 
flexes may  be  as  thoroughly  affected  in  one  disease  as  in 
the  other.  The  differential  diagnosis  can,  in  many  in- 
stances, be  made  only  after  a  close  observation  of  the  en- 
tire course  the  disease  has  taken.  (See  Chapter  on  Multi- 
ple Neuritis.) 

The  various  forms  of  progressive  muscular  dystrophies 
may  occasionally  be  mistaken  for  poliomyelitis  and  vice 
versa.  In  cases  that  are  seen  years  after  the  onset  of 
the  trouble,  the  initial  history  of  the  case  will  often  be 
ah  important  guide  to  diagnosis.  If  a  case  of  typical  mus- 
cular dystrophy  is  seen  during  the  stage  of  atrophy,  a  su- 
perficial inspection  of  the  case  may  suggest  an  old  polio- 
myelitis ;  but  in  the  progressive  dystrophies  the  atrophy 
affects  an  entire  limb  rather  than  single  groups,  or  if  it  has 
spread  to  several  extremities  it  is,  as  a  rule,  much  more 
general  than  in  cases  of  poliomyelitis,  while  in  the  latter 
the  electrical  changes  are,  as  a  rule,  much  more  complete 
than  in  cases  of  progressive  dystrophies. 

The  Charcot-Marie  type  (Tooth,  Hoffmann,  myself  and  others)  must  be 
differentiated  from  subacute  poliomyelitis. 

In  the  year  1900  Oppenheim  described  a  condition  observed  in  very  young 
children  which  he  designated  as  "  Myatonia  congenita."  The  symptoms 
bear  close  resemblance  to  those  of  poliomyelitis  anterior.  The  legs  and  pos- 
sibly the  arms  of  the  child  thus  afflicted  are  limp.  The  trunk  muscles  are 
rarely  affected.     The  deep  reflexes  may  be  diminished  or  lost,  but  the  ex- 


INFANTILE   SPINAL   PARALYSIS.  267 

tremities  are  not  as  completely  paralyzed  or  even  as  paretic  as  at  first  sight 
they  would  seem  to  be.  The  electrical  behavior  of  the  muscles  may  be  more 
or  less  altered.  There  is,  of  course,  no  history  of  an  acute  onset,  and  the 
entire  state  bears  the  earmarks  of  a  condition  of  defective  development. 

I  have  on  many  occasions  called  attention  to  the  muscular  weakness 
associated  with  rickets  and  which  may  easily  be  confounded  with  poliomye- 
litis anterior. 

PROGNOSIS. — Altogether  too  gloomy  a  prognosis  is  gen- 
erally given  in  poliomyelitis.  This  is  based  upon  the 
fact  that  some  palsy  always  remains,  but  the  actual  res- 
idue of  palsy  may  be  so  slight  that  one  should  be  care- 
ful not  to  depress  the  hopes  of  parents  and  patient.  Above 
all,  there  is  no  need  of  predicting  that  the  child  will  re- 
main a  hopeless  cripple  for  life.  There  is  no  telling  at  the 
outset  of  the  disease  to  what  extent  the  retrogression  may 
take  place  ;  but,  of  course,  the  more  widely  distributed  the 
paralysis  is  at  the  beginning  the  larger  the  remaining  palsy 
is  apt  to  be,  although  some  cases  which  begin  in  very 
stormy  fashion  exhibit  more  progress  than  those  which  be- 
gin less  violently.  Cases  in  which  but  a  few  muscles  are 
paralyzed  at  the  start  often  recover  with  very  little  per- 
manent injury.  Very  little  change  need  be  expected  in 
the  first  few  weeks  of  the  disease,  but  there  is  reason  to 
hope  that  those  parts  which  show  any  improvement  within 
the  first  few  weeks  or  months  after  the  onset  of  the  disease 
will  recover  power  before  long,  and  only  those  parts  will 
remain  permanently  paralyzed  which  after  months  show  no 
signs  of  improvement.  Muscles  which  are  paralyzed,  but 
which  exhibit  slight,  or  no  changes  of  electrical  reaction, 
may  be  regarded  in  a  hopeful  light,  and,  on  the  other  hand, 
those  which  very  soon  after  the  onset  of  the  palsy  exhibit 
distinct  reaction  of  degeneration,  and  for  months  afterward 
show  no  sign  of  change  in  this  respect,  are  apt  to  be  per- 
manently paralyzed.  The  more  complete  the  wasting  of 
the  muscles,  the  less  likely  these  muscles  are  to  recover ; 
and  if  contractures  form  in  the  opposing  groups  permanent 
disability  is  the  probable  result,  but  even  such  disability 
can  often  be  remedied  by  surgical  procedures. 

The  prognosis  as  regards  life  is,  with  few  exceptions,  en- 
tirely favorable.     Cases  that  end  fatally  are  apt  to  do  so 


268  THE  NERVOUS  DISEASES   OF   CHILDREN. 

within  the  first  few  weeks  of  the  disease.  But  parents  are 
often  most  grateful  for  the  assurance  that  if  the  child  sur- 
vives, however  great  the  paralysis  may  be,  its  mental  de- 
velopment will  in  nowise  be  impaired.  Hirsch,  Cassirer, 
and  others  have  shown  that  individuals  who  had  poliomye- 
litis may  develop  amyotrophies  or  dystrophies. 

Treatment. — During  the  acute  stage  of  an  anterior 
poliomyelitis  the  general  condition  only  should  be  treated 
and  little  attention  need  be  paid  to  the  paralysis.  The  child 
should  be  kept  in  a  quiet  room,  mild  antipyretic  measures 
may  be  employed,  such  as  small  doses  of  phenacetin,  of  anti- 
pyrin,  of  the  salicylates,  and  the  like.  In  the  earlier  stages 
cold  applications,  or  mild  counter-irritation  over  that  part 
of  the  spine  which  is  involved  in  the  given  case,  are  quite  in 
order ;  and  the  attempt  should  be  made  to  limit  the  spread 
of  the  inflammation  by  the  administration  of  small  doses  of 
bromide  and  of  ergot  (a  few  drops  of  the  fluid  extract). 
Iodides  and  other  drugs  I  have  found  to  be  utterly  ineffi- 
cient. The  child  should  during  this  period  be  carefully  fed, 
and  the  bowels  should  be  thorougly  purged  by  the  use  of 
small  but  sufficient  doses  of  calomel.  After  the  acute  stage 
is  passed  the  paralyzed  muscles  demand  treatment.  Elec- 
tricity and  massage  are  the  most  effective  therapeutic 
measures.  Avoid  electrization  of  the  spine  ;  first,  because 
it  is  not  at  all  certain  that  the  electric  current  reaches  the 
spinal  cord,  and  secondly,  because  the  use  of  strong  cur- 
rents makes  the  child  extremely  restless  and  may  do  more 
harm  than  good.  In  the  treatment  of  paralyzed  muscles  an 
important  use  of  the  electric  current  is  to  exercise  muscles 
which  are  no  longer  subject  to  the  will ;  it  supplies,  in 
other  words,  a  convenient  form  of  gymnastics.  The  cur- 
rent may,  in  addition,  improve  the  state  of  the  paralyzed 
and  atrophied  muscles,  but  whether  or  not  it  increases  the 
conductive  powers  of  paralyzed  nerve  and  muscle  I  am  not 
willing  to  assert.  Since  we  wish  to  make  the  muscles  con- 
tract, the  only  form  of  current  that  is  serviceable  is  that 
form  to  which  the  muscle  will  respond.  If  the  reaction  of 
degeneration  is  complete  the  faradic  current  is  quite  useless, 
and  if  the  diagnostic  tests  have  shown  that  the  muscles  re- 
spond to  the  anode  better  than  to  the  cathode,  exercise  by 


INFANTILE   SPINAL  PARALYSIS.  269 

anodal  opening  or  closing  of  the  current  is  the  only  proper 
method. 

This  should  be  done  in  sittings  of  ten  to  fifteen  minutes  once  or  twice  a 
day,  and  that  strength  of  current  should  be  employed  which  is  sufficient  to 
produce  mild  contractions.  Excessive  contractions  are  not,  called  for  and 
help  to  increase  the  difficulties  of  application.  If  a  muscle  responds  to  the 
faradic  current,  however  slightly,  that  current  should  be  employed  together 
with  the  galvanic,  and  it  has  been  my  habit,  even  in  the  more  severely  par- 
alyzed cases  of  poliomyelitis,  to  make  occasional  tests  with  the  faradic  cur- 
rent, first,  in  order  to  determine  whether  there  is  any  sign  of  improvement  in 
any  group  of  muscles  that  have  been  paralyzed,  and,  secondly,  to  give  such 
muscles  the  benefit  of  both  currents.  If  the  muscles  react  at  all  to  both  cur- 
rents, both  may  be  employed  in  one  and  the  same  sitting,  or  given  in  alter- 
nate sittings.*  I  am  a  thorough  believer  in  the  good  influence  that  massage 
has  upon  atrophied  and  palsied  muscles.  It  helps  undoubtedly  to  keep  up 
the  nutrition  of  such  parts,  and  in  cases  in  which  there  is  an  incipient  ten- 
dency to  contracture  such  tendency  may  be  overcome  by  the  proper  use  of 
massage.  But  this  should,  if  possible,  be  intrusted  to  skilled  manipulators 
and  not  to  the  mother  of  the  child  or  to  a  nurse,  whose  "  rubbings  "  are,  as  a 
rule,  wholly  ineffective.  Passive  movements  are  entirely  in  order,  and  some 
good  results  have  been  attained  by  the  regular  use  of  Swedish  movements, 
such  as  are  given  by  trained  rubbers  or  by  a  regular  system  of  treatment  in 
a  well-equipped  Zander  institute. 

During  the  chronic  stages  of  poliomyelitis  orthopedic 
measures  should  be  employed  without  reserve.  If  the  con- 
tractures have  persisted  for  years  they  will  not  disappear 
without  treatment.  Tenotomies  are  followed  by  results 
fully  as  favorable  as  those  in  chronic  ocular  palsies.  The 
general  condition  of  the  child  and  the  condition  of  the 
paralyzed  muscles  improves  markedly  after  the  orthopedic 
surgeon  has  done  his  work,  and  such  tenotomies  need  not 
be  restricted  to  the  tendon  Achillis,  but  many  of  the  other 
muscles  which  are  in  a  state  of  contracture  can  as  well  be 
similarly  treated.  The  application  of  splints  according  to 
the  best  orthopedic  principles  is  also  of  great  assistance  to 
the  child,  and  this,  too,  should  not  be  delayed  too  long,  for 
it  is  far  better  to  have  a  child  walk  in  splints  than  to  have 
ugly  deformities  of  the  joints  develop  which  may  cripple 
him  for  life.  Since  I  have  recognized  the  truth  of  these 
principles  I  have  had  the  satisfaction  of  seeing  children, 

*  I  have  seen  no  reason  to  advocate  static  or  high  tension  currents  in  cases  of 
poliomyelitis. 


270  THE   NERVOUS  DISEASES   OF  CHILDREN. 

and  even  very  young  children,  walk  within  a  few  months 
after  the  development  of  a  poliomyelitis,  while  in  former 
years  such  children  were  compelled  to  be  carried  about  in 
the  arms  of  a  nurse  or  to  be  wheeled  about  in  chairs.  I  am 
satisfied  that  few  cases  of  poliomyelitis  are  so  severe  that 
much  cannot  be  done  by  the  proper  application  of  ortho- 
pedic measures.  In  cases  in  which  the  joints  are  entirely  use- 
less on  account  of  the  complete  atrophy  of  the  muscles,  the 
operation  for  arthrodesis,  as  suggested  by  Wolff  and  others, 
may  be  resorted  to.  In  this  way  a  leg  that  would  other- 
wise be  entirely  useless  may  be  made  to  subserve  the  func- 
tion of  standing  and  walking,  though  of  course  the  station 
and  the  gait  of  the  person  will  always  be  far  from  normal. 
Many  years  ago  Nicoladoni  suggested  the  transplantation 
of  tendons  in  these  cases.  The  "  method  "  was  revived  by 
Gluck,  Lange,  Vulpius,  and  Hoffa.  Townsend  and  Gerster 
have  operated  upon  a  number  of  my  patients.  The  princi- 
ple is  a  simple  one :  Split  the  tendon  of  the  overacting 
muscle,  and  transplant  it  into  the  underacting ;  but  make 
sure  that  the  "  overacting  "  muscles  are  in  good  condition 
and  that  the  "underacting"  muscles  are  not  completely 
wasted.  To  correct  the  position  of  flaccid  limbs  good  can 
be  done  by  shortening  of  the  tendons  as  practised  by  Hoffa. 
Much  is  to  be  expected  of  the  orthopedic  surgeon  in  this 
field.  Spiller,  Young,  and  Frazier  have  attempted  in  cases 
of  poliomyelitis  in  which  only  a  few  muscles  were  paralyzed 
to  improve  the  condition  of  the  limb  by  nerve-transplanta- 
tion. (See  Journal  of  Nervous  Mental  Disease,  1903,  p.  369; 
also  Journal  of  Am.  Med.  Ass.,  1905,  p.  169.) 

SUBACUTE   ANTERIOR    POLIOMYELITIS. 

Subacute  anterior  poliomyelitis  is  practically  a  mere  variety  of  the  acute 
form  of  the  disease,  and  for  that  reason  needs  but  little  special  mention.  The 
entire  difference  between  the  two  diseases  is  in  the  mode  of  onset,  and  ac- 
cording to  our  present  views  of  the  character  and  origin  of  acute  spinal  palsy, 
the  subacute  variety  necessarily  implies  a  milder  form  of  infection  than  in  the 
cases  with  a  more  acute  and  more  violent  beginning.  The  difference  in  the 
symptoms  is  also  confined  entirely  to  the  difference  in  the  manner  of  onset 
and  the  manner  in  which  the  paralysis  is  developed.  In  these  cases  of  the 
subacute  variety  we  find  that  the  disease  comes  on  very  gradually.     The 


INFANTILE  SPINAL  PARALYSIS.  271 

child  is  ill  at  ease  for  some  days  or  weeks,  complains  of  weariness  in  walking, 
of  pains  in  the  joints  and  muscles.  After  some  little  while  a  decided  paresis 
of  one  or  more  groups  of  muscles  is  observed,  generally  in  the  lower  ex- 
tremities. This  increases,  and  after  a  week  or  more  a  distinct  paralysis  is 
developed.  The  paralysis  then  increases  in  the  affected  muscles,  and  is  apt 
to  spread  somewhat  after  the  fashion  of  a  progressive  muscular  atrophy  from 
one  group  of  muscles  to  another.  There  is,  therefore,  a  progression  in  these 
cases  at  the  start,  but  the  limit  is  very  soon  reached,  and  from  this  time  on  a 
retrogression  again  sets  in,  though  not  in  such  a  marked  degree  as  in  the 
acute  cases.  The  fact  that  the  retrogression  occurs  after  a  given  period  ot 
time  will  dispel  all  fears  as  regards  the  possibility  of  a  progressive  form  of 
muscular  atrophy. 

The  differential  diagnosis  between  these  diseases  is  difficult,  and  cases  are 
confounded  easily  with  forms  of  progressive  muscular  atrophy,  particularly  of 
the  peroneal  type,  and  with  cases  of  chronic  neuritis ;  but  the  points  of  dif- 
ferential diagnosis  which  were  given  between  these  diseases  and  the  acute 
form  of  anterior  poliomyelitis  will  also  help  us  in  arriving  at  a  correct  diag- 
nosis of  the  subacute  variety. 

Treatment. — The  treatment  should  be  conducted  on  the  same  princi- 
ples as  were  enunciated  in  the  preceding  discussion  on  acute  spinal  palsy.  In 
those  cases  in  which  the  original  disease  is  not  nearly  so  violent  as  in  the 
acute  form  even  more  can  be  hoped  from  an  early  application  of  therapeutic 
measures,  but  it  should  be  remembered  that  the  natural  course  of  the  disease 
tends  much  more  to  recovery  than  does  the  acute  form,  so  that  tenotomies 
and  other  surgical  procedures  should  be  delayed  until  the  disease  has  become 
entirely  stationary,  and  there  is  no  reason  to  think  that  further  spontaneous 
recovery  will  take  place. 


CHAPTER  XV. 

ACUTE   MYELITIS. 

Myelitis,  or  inflammation  of  the  spinal  cord,  has  been 
made  to  cover  a  multitude  of  diagnostic  sins,  both  in  the 
adult  and  in  the  child.  If  we  subdivide  the  cases  accord- 
ing to  the  mode  of  onset,  we  may  distinguish  between  an 
acute,  a  subacute,  and  a  chronic  form  ;  and  if  the  classifica- 
tion is  based  on  the  origin  of  the  myelitis  we  have  an  idio- 
pathic, a  traumatic,  a  tubercular,  a  syphilitic,  and  an  imjSA- 
tious  varietyT^As  the  symptoms  are  very  much  the  same, 
whatever  the  original  cause  of  the  disease  may  be,  it  will 
be  better  to  describe  the  characteristic  features  of  acute 
myelitis,  which  occurs  frequently  enough  in  children  to  de- 
mand special  study. 

Symptoms. — The  symptoms  in  a  given  case  will  vary  ac- 
cording to  the  site  of  the  inflammation,  and  the  intensity  of 
the  process.  Its  clinical  features  will  depend  upon  the 
amount  of  cord  tissue  involved.  In  such  cases  more  than 
in  any  others  an  accurate  knowledge  of  the  functions  con- 
nected with  each  segment  of  the  cord  is  of  importance  if  a 
satisfactory  diagnosis  is  to  be  made.  In  every  case  of  com- 
plete transverse  myelitis,  at  whatever  level  the  area  of  in- 
flammation may  be,  motion  and  sensation  are  chiefly  af- 
fected, the  reflexes  are  disturbed,  and  the  functions  of  the 
bladder  and  rectum  are  deranged.  Bilateral  paralysis  (para- 
plegia) is  the  natural  result  of  a  myelitis.  If  the  lesion  is  in 
the  cervical  portion  of  the  cord,  both  upper  and  both  lower 
extremities  will  be  paralyzed  ;  the  first  because  the  very 
parts  which  are  most  intimately  connected  with  the  motion 
of  the  upper  extremities  are  destroyed  ;  and  the  lower  ex- 
tremities are  involved  because  the  fibres  going  to  them  are 
interrupted  at  the  site  of  the  lesion.     The  bilateral  character 


ACUTE   MYELITIS. 


273 


of  a  palsy,  is  after  all,  the  one  symptom  which  points  more 
frequently  to  a  spinal  lesion  than  any  other. 

Almost  the  only  exceptions  to  this  rule  are  those  cases  in  which  a  multi- 
ple neuritis,  an  ascending  Landry's  paralysis,  or  double  cerebral  lesions 
give  rise  to  a  bilateral  form  of  palsy. 

If  the  lesion  is  in  the  cervical  portion  of  the  spinal  cord, 
the  paralysis  of  the  upper  extremities  will  be  of  a  flaccid 
order;  the  paralysis  of  the  lower  extremi- 
ties will  be  spastic  in  character.  Anaesthesia 
will  be  present  in  the  four  extremities  and 
in  the  trunk  to  the  level  of  the  diseased  seg- 
ments; pupillary  symptoms,  unilateral  blush- 
ing (due  to  lesion  of  the  sympathetic),  and 
paralysis  of  the  diaphragm  are  present  in 
some  cases.  If  the  lesion  is  in  the  lumbar 
portion  of  the  cord,  the  paralysis  is  re- 
stricted to  the  lower  extremities  and  will 
be  of  a  flaccid  character,  with  more  or  less 
It  the^tesion  is  in  the  cervical  or 


atropFr 

dorsalportion  of  the  cord,  and  the  lumbar 
portion  is  entirely  free  from  disease,  the 
paraplegia  of  the  lower  extremities  is  of 
the  spastic  order,  and  ThTreason  of  this  can 
be  easily  understood  if  we  recall  the  fact 
that  after  a  transverse  lesion  in  any  por- 
tion of  the  spinal  cord  the  lateral  columns 
will  degenerate  downward  from  that  level 
(Fig.  64),  and  that  such  degeneration  of  the 
lateral  columns  in  the— presence  of  normal 
gray  matter  of  the  lumbar  segments  will 
produce  a  spastic  form  of  paralysis  with 
rigidities  and  contractures.  Taking  all 
cases  of  myelitis,  the  largest  number  affect  the  dorsal  re- 
gion ;  and  in  these  the  arms  go  free,  as  their  spinal  centres 
are  above  the  site  of  the  lesion,  but  the  lumbar  segments 
are  affected  by  secondary  degeneration. 

Sensation  is  impaired  at  a  very  early  period  of  the  dis- 
ease, often  from  the  first  moment  of  onset.  In  fact,  in  the 
cases  of  traumatic  origin  loss  of  power  and   loss  of  sensa- 


Fig.  64.—  Seconda- 
ry Ascending  and 
Descending  De- 
generation follow- 
ing a  Transverse 
Lesion  in  the  Up- 
per Dorsal  Cord. 
(Struempell.) 


274  THE  NERVOUS  DISEASES   OF   CHILDREN. 

tion  are  effected  almost  instantaneously,  -so  that  a  person 
thus  afflicted  feels  not  only  that  his  legs  are  powerless,  but 
that  they  appear  to  be  dead  as  well.  Anaesthesia  pyists  in 
all  the  parts  that  are  supplied  by  nerves  coming  off  below 
the  site  of  the  spinal  lesion,  and  in  the  typical  cases  all 
forms  of  sensation  are  equally  involved.  Thus  we  gener- 
ally find  that  a  spastic  paraplegia  is  associated  with  the 
loss  of  touch  sense,  of  pain  sense,  of  thermal  sense,  and 
of  muscular  sense  in  the  affected  parts.  As  the  anaesthesia 
is  strictly  dependent  upon  the  conduction  of  impulses  in- 
ward through  the  posterior  roots  of  the  cord,  the  extent  of 
the  anaesthetic  area  will  naturally  point  to  the  upper  limit 
of  disease.*  At  the  upper  limit  of  the  anaesthetic  area  a 
small  zone  of  hyperesthesia,  as  a  rule,  begins.  This  is 
evidence  of  the  fact  that  in  the  segment  supplying  the  hy- 
peraesthetic  area  there  is  a  condition  of  irritation  affecting 
the  posterior  spinal  root-fibres,  but  not  the  chief  morbid  and 
destructive  process.  Above  the  hyperaesthetic  area  sensa- 
tion may  be  expected  to  be  entirely  normal.  In  other  cases, 
instead  of  a  distinct  hyperaesthesia  we  have  a  girdle  sensa- 
tion, which  also  marks  the  level  between  the  normal  and 
diseased  segments. 

The  state  of  the  reflexes  helps  us  also  to  determine  the 
area  involved.  If  the  lesion  is  in  the  cervical  region  all 
the  reflexes  of  the  upper  extremities  are  destroyed,  those 
in  parts  below  will  be  exaggerated.  If  the  lesion  is  in  the 
dorsal  region  the  reflexes  connected  with  these  segments, 
such  as  the  abdominal  and  epigastric  reflexes,  will  be  lost 
and  the  lower  reflexes  will  be  increased.  If  the  lesion  is  in 
the  lumbar  region  the  knee-jerk  will  be  lost  and  the  ankle 
clonus_wiU^bo  absent  also.  In  some  cases  in  which  there 
is  a  very  narrow  band  of  inflammation  these  reflexes  may 
behave  differently  and  may  give  one  a  direct  clew  as  to  the 
exact  extent  of  spinal  inflammation.  Thus  in  one  case  un- 
der my  observation  the  knee-jerk  was  lost,  but  the  ankle 
clonus  was  present.  This,  taken  in  conjunction  with  a  line 
of  anaesthesia  showing  an  involvement  of  the  lower  dorsal 
segment,  proved  that  the' upper  lumbar  region  was  slightly 
involved,  but  that  the  lower  lumbar  and  sacral  segments 

*  The  reader  is  advised  to  consult  special  tables  on  Spinal  Localization. 


ACUTE  MYELITIS.  275 

were  not  directly  implicated  by  the  disease.  Since  dorsal 
myelitis  is  the  most  frequent  form,  it  is  also  common  to 
have  exaggeration  of  the  reflexes  associated  with  spastic 
contractures  of  the  legs. 

The  electrical  reactions  will  vary  according  to  the  seg- 
ments involved.  In  cases  of  cervical  lesion  the  reaction  of 
degeneration  will  be  found  present  in  many,  if  not  all  of  the 
muscles  of  the  upper  extremities-,  at  least  after  the  lapse  of 
a  few  days  or  weeks  ;  those  in  the  lower  extremities  will 
remain  unaltered.  But  if  the  lesion  is  in  the  lumbar  seg- 
ments the  reaction  of  degeneration  will  be  present  in  the 
muscles  supplied  by  nerves  coming  off  from  the  diseased 
area.  In  cases  of  cervical  and  dorsal  myelitis  we  may, 
therefore,  expect  entirely  normal  electrical  reactions  in  the 
lower  extremities,  however  thoroughly  paralyzed  these 
parts  may  be. 

A  few  other  symptoms  must  be  noted  which  are  ex- 
tremely characteristic  of  acute  myelitis  and  often  serve  to 
reveal  the  disease  when  other  symptoms  in  the  case  have 
left  the  diagnosis  in  doubt.  In  all  such  cases  of  myelitis,  in 
whatever  region  the  lesion  may  be,  the  vesical  and  rectal 
reflexes  are  disturbed  or  completely  abolished.  The  re- 
sult of  this  is  retention  of  urine,  with  possible  overflow,  or 
constant  dribbling  of  urine,  and  either  retention  of  stool  or 
involuntary  defecation.  Since  the  centres  for  these  reflexes 
are  in  the  lowest  portion  of  the  cord,  and  every  part  of  the 
cord  must  be  intact  if  such  sensation  is  to  be  conducted  to 
the  higher  centres,  we  can  understand  why  these  symptoms 
should  be  present,  whether  the  lesion  be  in  the  lumbar,  in 
the  dorsal,  or  in  the  cervical  segments.  Loss  of  sexual 
function  is  hardly  to  be  mentioned  in  cases  of  myelitis  in 
children  ;  but  priapism,  due  to  irritation  of  the  spinal  sexual 
centre,  is  not  infrequently  present  in  children,  and  is  some- 
times a  very  annoying  symptom.  Involuntary  spasmodic 
twitchings  occur  in  nearly  every  form  of  myelitis.  I  know 
of  no  symptom  which  is  on  the  whole  more  characteristic 
of  spinal  lesions,  and  which  often  serves  as  a  differential 
symptom  between  supposed  functional  and  spinal  paralysis. 
This  spasmodic  cramp  is  evidently  due  to  an  irritation  of 
the  normal  ganglion   cells,  and  the  irritability  is  often  so 


276  THE  NERVOUS  DISEASES   OF   CHILDREN. 

great  that  the  mildest  form  of  sensory  impulse  is  sufficient 
to  elicit  such  involuntary  spasms.  Under  these  circum- 
stances the  mere  touch  of  the  paralyzed  part — of  a  toe,  for 
instance — is  sufficient  to  produce  contraction  of  the  entire 

limb. 

Trophic  disturbances  are  exceedingly  common  and 
much  to  be  feared.  Bed-sores  are  easily  developed  in  all 
parts  on  which  pressure  is  exerted  :  under  the  shoulder- 
blades,  over  the  sacrum,  on  the  hips,  and  even  over  the 
internal  malleoli  or  on  the  inner  surfaces  of  the  knees  and 

thighs in  short,  wherever  parts  touch,  are  pressed  upon, 

or  are  pressed  against  each  other.  In  the  sacral  region  the 
constant  wetting  of  the  bed  and  the  uncleanliness  of  the 
patient  may  increase  the  danger  and  size  of  bed-sores ;  and 
while  it  is  true  that  such  bed-sores  may  occur  even  without 
any  external  irritation,  they  are  greatly  aggravated  by  the 
dribbling  of  urine  or  the  involuntary  evacuation  of  the 
bowels.  These  bed-sores  begin,  as  a  rule,  as  a  mere  redden- 
ing of  the  skin ;  the  epidermis  is  soon  worn  away,  the 
cutis  is  bared,  this  too  disappears,  and  gradually  the  ulcer 
may  eat  away  all  the  subjacent  parts  until  the  bone  itself  is 
laid  bare,  provided  the  patient  lives  a  sufficient  period  of 
time. 

Every  form  of  acute  myelitis  may  be  accompanied  by 
fever,  which  may  vary  between  ioo°  and  1040  F.  and  higher. 
The  fever  is  due  to  the  myelitic  process,  or  the  infection, 
but  is  often  increased,  and  sometimes  maintained  altogether, 
by  the  complicating  conditions  of  myelitis.  Such  compli- 
cations are  deep  bed-sores  with  the  absorption  of  putrid 
matter  and  the  danger  of  phlebitis  ;  furthermore,  the  oc- 
currence of  cystitis  and  pyelonephritis,  which  are  not  un- 
common. In  cases  of  myelitis,  in  which  fever  suddenly 
increases,  with  chills,  with  deep  remissions  and  sudden  ex- 
acerbations, the  probability  of  this  fever  being  due  to  some 
pyasmic  process  is  very  great  indeed.  As  soon  as  the  dis- 
ease has  passed  the  acute  stage  the  fever  lessens  and  the 
temperature  will  remain  entirely  normal  until  some  com- 
plicating condition  is  established. 

Whenever  a  majority  of  the  above  symptoms  are  pres- 
ent, the  diagnosis  of  an  acute  (transverse)  myelitis  can  safely 


ACUTE  MYELITIS.  277 

be  made.  The  modification  of  the  symptoms  if  the  mye- 
litis is  not  complete,  or  if  it  is  subacute  and  chronic  can  be 
easily  inferred  from  the  preceding  account.  The  question 
of  greatest  interest,  in  every  case,  is  to  determine  what  the 
origin  of  the  myelitis  may  be.  Idiopathic  myelitis  is,  ac- 
cording to  our  present  notions,  scarcely  conceivable,  and 
here,  as  in  so  many  other  instances,  it  is  better  to  say  mye- 
litis from  unknown  cause  than  to  assume  that  in  some 
cases  the  origin  is  truly  spontaneous.  In  cases  occurring 
in  children,  without  known  cause,  I  am  inclined  to  suspect 
slight  traumatism.  ■ 

A  child  of  eight  years,  a  healthy,  beautiful  girl,  while  walking  on  a  coun- 
try road,  had  a  desire  to  urinate.  Her  mother  urged  her  to  do  so  on  the 
road.  The  child  hurried  to  one  side,  and  in  attempting  to  place  its  right 
foot  on  a  stone  not  more  than  a  few  inches  in  height,  lost  its  bal- 
ance and  struck  on  the  middle  of  the  back.  The  child  experienced  pain  at 
once,  but  was  able  to  walk  some  little  distance ;  soon  the  power  of  its  legs  di- 
minished; it  had  to  be  carried,  was  put  to  bed,  and  within  twenty-four  hours 
had  developed  a  most  pronounced  form  of  acute  transverse  myelitis.  After 
three  days  anaesthesia  was  complete  up  to  the  umbilicus.  The  paralysis 
was  absolute  in  the  lower  extremities.  There  was  retention  of  urine  and 
faeces,  bed-sores  were  developed,  and  the  child  died  from  these  complicating 
conditions  within  three  weeks  after  the  accident. 

In  other  cases  the  spinal  injury  is  much  more  severe,  and 
a  complete  destruction  of  the  cord  may  be  the  result  of  in- 
jury to  the  spinal  column.  From  the  effect  of  concussion 
alone,  without  actual  destruction  of  the  bony  parts  surround- 
ing the  cord,  an  acute  myelitis  may  result.  The  myelitis 
which  occurs  in  connection  with  tumors  of  the  cord,  with 
tubercular  affections  of  the  meninges,  need  not  be  separately 
considered,  as  it  constitutes  merely  a  part  of  the  more  seri- 
ous disease.  The  two  forms  of  myelitis  which  are  most 
common  in  children  are  those  due  to  Pott's  disease  and  to 
syphilitic  infection  ;  but  both  these  forms  are  so  distinct 
and  so  important  that  they  deserve  special  consideration. 

Acute  myelitis  does,  however,  occur  in  connection 
with  other  acute  infectious  diseases,  such  as  typhoid,  scar- 
let fever,  small-pox,  and  the  like.  Some  of  these  cases  of 
supposed  myelitis  have  probably  been  cases  of  multiple 
neuritis.     Rheumatic  or  atmospheric  influences  (refrigera- 


278  THE   NERVOUS  DISEASES   OF   CHILDREN. 

tion),  as  a  direct  cause  of  myelitis  in  the  child  and  in  early 
youth,  should  be  considered  duly.  A  young  girl,  of  about 
sixteen  years  of  age,  was  brought  to  me  from  the  South, 
with  the  following  history:  On  a  very  warm  day  in  early 
spring  she  had  taken  a  warm  bath  and  had  sat  down  at  an 
open  window  in  the  evening,  immediately  after  the  bath, 
with  nothing  but  a  light  chemise  to  cover  her  body.  She 
sat  there  for  hours  and  fell  soundly  asleep.  The  next  morn- 
ing she  experienced  considerable  difficulty  in  the  use  of 
both  upper  extremities.  Within  a  few  days  these  became 
absolutely  paralyzed,  and  the  legs  at  the  same  time  grew 
stiff  and  motionless.  She  has  since  that  time,  a  period  of 
fully  sixteen  years,  been  suffering  from  the  effects  of  this 
myelitis,  and  even  now  presents  an  atrophic  form  of  paral- 
ysis of  the  right  upper  extremity,  with  slight  involvement 
of  the  left,  and  with  a  complete  spastic  paralysis  of  both 
lower  extremities,  with  incontinence  of  urine  and  fseces,  and 
with  considerable  disturbances  of  sensation,  though  the  lat- 
ter have  been  recovered  from  very  much  more  than  has 
been  the  paralysis  or  the  atrophy. 

The  infectious  origin  of  myelitis  has  been  demonstrated.  Streptococci 
and  staphylococci  have  been  found  by  Marinesco,  Tooth,  Russell,  and  others. 
The  author  has  reported  a  case  occurring  in  a  young  girl  in  association  with 
multiple  streptococci  abscesses.  Fiirstner  found  pneumococci  in  a  myelitic 
focus.  Strumpell  lays  great  stress  on  the  toxic  rather  than  the  infectious 
origin  of  myelitis. 

Pathology  and  Morbid  Anatomy.  —  The  delicate 
structure  of  the  spinal  cord  seems  peculiarly  liable  to  in- 
flammatory disease.  The  cervical  and  lumbar  enlargements 
of  the  cord  are  less  frequently  the  seat  of  such  inflammation 
than  the  dorsal  portion.  The  reason  of  this  is  not  easy  to 
explain,  although  it  must  in  all  probability  be  sought  in  the 
peculiarity  of  the  blood-supply. 

There  can  be  little  doubt  that  if  the  blood-supply  of  the  cord  is  interfered 
with,  necrotic  softening  follows  as  in  the  case  of  other  organs.  In  the  case 
of  one  form  of  myelitis — that  due  to  specific  disease — the  relation  of  the  my- 
elitis to  disease  of  the  blood-vessels  can  be  clearly  demonstrated.  Under 
these  conditions  the  smaller  vessels  are  blocked  by  thrombi,  and  the  result  of 
this  obstruction  is  a  necrotic  softening  of  the  surrounding  parts.  In  the  cases 
of  traumatic  myelitis  the  earliest  changes  are  due  to  mechanical  injury  of  the 
part,  with  compression  of  the  delicate  structures  of  the  cord  by  effusion  of 


ACUTE  MYELITIS.  2J() 

blood,  and  to  the  necrosis  that  follows  such  compression.  It  is  more  difficult  to 
explain  the  exact  manner  in  which  myelitis  is  developed  in  the  cases  of  a  toxic 
character,  unless  we  suppose  that  the  chemically  altered  states  of  the  blood 
produce  coagulation  of  the  blood  and  obstruction  of  blood-vessels,  with  the 
same  result  as  in  those  cases  in  which  these  conditions  are  brought  about  by 
other  disease  of  the  blood-vessels  themselves.  The  origin  of  myelitis  from 
refrigeration  and  rheumatic  influences  in  general  cannot  be  satisfactorily  ex- 
plained in  this  way,  nor  can  any  other  plausible  explanation  be  substituted. 
The  myelitis  which  results  from  mere  concussion  without  any  visible  anatom- 
ical changes  must  be  explained  on  the  supposition  that  minute  changes  in 
the  gray  and  the  white  matter  are  present,  such  as  were  found  in  the  spinal 
cords  of  animals  experimented  on  by  Schmauss. 

If  the  exacts  mod£_of  origin  of  various  forms  of  myelitis 
is  still  unknown,  the  morbid  anatomy  is  no  longer  a  matter 
of  doubt.  As  for  the  macroscopical  appearance  of  myelitis, 
the  cord  so  diseased  is  generally  surrounded  by  the  hyper- 
semic  meninges,  and  the  cord  itself,  if  inflammation  is  recent, 
may  appear  to  be  congested  and  slightly  swollen.  The  dis- 
tinction between  the  white  and  the  gray  matter  is  often  not 
so  marked  as  in  the  normal  cord.  There  is,  furthermore,  a 
change  in  the  consistence  of  the  cord,  which  may  be  either 
slightly  softer  than  normal  or  else  so  diffluent  that  as  soon 
as  the  pia  is  cut  open  the  cord  flows  out  like  creamy  pus. 
This  is  the  condition  often  found  on  post-mortem  exami- 
nation ;  in  all  probability  the  cord  is  not  nearly  so  soft 
during  life,  but,  like  other  necrosed  tissue,  softens  consid- 
erably immediately  after  death.  In  acute  myelitis  minute 
hemorrhages  are  extremely  frequent,  and  the  altered  cord 
may  present  the  appearance  of  red  softening.  There  is 
every  degree  of  change  between  simple  red  softening  and 
the  condition  of  hemorrhagic  myelitis  in  which  the  extrava- 
sation of  blood,  being  considerable,  for  the  time  obscures 
all  other  changes.  If  the  blood  has  been  exuded  for  some 
time  before  death  its  color  may  have  changed,  and  the  con- 
dition be  that  of  yellow  softening.  We  also  may  discern  a 
condition  of  white  softening  in  which  the  white  matter  has 
become  diffluent  without  any  admixture  of  blood.  If  ex- 
amined microscopically  the  cord  is  found  to  contain  ample 
evidence  of  inflammatory  changes.  Among  these  are  dilated 
blood-vessels,  with  leucocytes,  granules  of  myelin,  and,  fur- 
thermore, bodies  well  known  as  corpora  amylacea.      The 


Fig.  65. — Acute  Disseminated  Myelitis.      F,  small  foci  containing  round  cells  generally 
clustered  about  blood  vessels.       van  Gieson  stain  (after  Oppenheim). 


Fig.  66.— One  of  the  foci  (F  of  Fig.    65)  more  highly  magnified  (after  Oppenheim). 

:     s 


L 


y& 


Fig.  67. — Swollen  and  disintegrating  cells  in  a  case  of  myelitis 
2S0 


ACUTE   MYELITIS.  2-Sl 

softened  tissue  also  contains  axis  cylinders  in  various  states 
of  disintegration. 

In  many  cases  of  diffluent  myelitis  these  changes  of  the  individual  ele- 
ments of  the  cord  are  the  only  ones  that  can  be  distinctly  made  out.  But  in 
the  parts  directly  surrounding  the  focus  of  most  intense  inflammation  further 
changes  can  be  made  out  after  proper  hardening  and  staining  with  the  various 
dyes.  On  such  sections  the  blood-vessels  will  be  found  dilated,  and  innumer- 
able leucocytes  can  be  seen  in  the  vicinity  of  such  vessels.  The  nuclei  of  the 
smaller  arteries  and  capillaries  will  be  found  to  be  enormously  increased  and 
in  a  state  of  proliferation.  The  sheath  of  the  blood-vessels  is  very  much 
distended,  blocked  in  part  by  the  coagulation  of  blood,  and  round  blood- 
corpuscles  may  even  he  found  in  the  adjacent  tissue. 

In  the  gray  substance  the  large  nerve-cells  are  swollen  and  granular, 
many  of  these  granules  showing  distinct  evidences  of  degeneration.  The  proc- 
esses of  the  cells  are  either  shrivelled  up  or  entirely  lost,  the  contour  of  the 
cells  less  distinctly  defined,  and  changed  from  the  polygonal  form  to  spher- 
ical or  oval-shaped  bodies.  The  neuroglia  of  the  gray  matter  will  appear 
denser  than  under  normal  conditions.  In  the  white  substance  similar 
changes  will  be  found  in  the  blood-vessels  and  in  the  interstitial  tissue.  The 
white  substance  often  has  a  distinctly  fibrous  appearance,  containing  many 
spider-cells  or  cells  of  Deiters.  The  white  nerve-fibres  themselves  undergo 
degeneration.  The  axis  cylinders  are  irregular,  swollen,  and  often  trans- 
versely divided.^ In  some  cases  the  entire  nerye-fibre  is  destroyed  or  disin- 
tegrated and  the  space  once  occupied  by  stich  fibres  is  left  vacant  or  occupied 
by  granular  matter.  In  some  cases  of  myelitis  the  nerve  elements  are  more 
intensely  affected  than  the  interstitial  tissue,  while  in  others  the  changes  in 
the  interstitial  tissue  are  the  more  prominent  feature  in  the  cross-section,  and 
the  nerve-tissue  has  evidently  been  destroyed  secondarily.  In  transverse 
myelitis  the  changes  may  be  distributed  equally  through  the  entire  cross-sec- 
tion ;  in  other  cases  the  changes  may  be  more  intense  in  the  gray  than  in  the 
white  matter,  and  in  some  more  intense  in  the  ventral  half  than  in  the  dorsal 
half.  In  cases  of  meningo-myelitis,  particularly  in  those  of  traumatic  or 
specific  origin,  the  most  marked  changes  are  near  the  periphery.  Here  the 
pia  will  appear  thickened  and  the  morbid  changes  can  be  traced  along  the 
connective  tissue  passing  from  the  pia  into  various  portions  of  the  cord.  If 
the  myelitis  is  of  the  disseminated  order,  small  foci  of  disease  may  appear  in 
various  portions  of  the  cross-section  and  in  various  segments  of  the  cord, 
intervening  parts  maintaining  a  tolerably  normal  appearance.  If  the  myelitic 
changes  are  most  prominent  in  the  vicinity  of  the  central  canal  and  the  parts 
surrounding  it,  we  speak  of  a  central  myelitis ;  but  it  is  rare  to  find  such 
central  myelitis  without  some  additional  symptoms  of  a  diffuse  inflammatory 
process.  The  nerve-roots  in  connection  with  the  inflamed  segments  are,  as 
a  rule,  altered,  and  will  present  appearances  somewhat  similar  to  those  found 
in  the  white  matter  of  the  cord.  The  vessels  are  dilated,  the  nuclei  and  the 
tissues  about  these  vessels  exhibit  various  degrees  of  proliferation,  the  myelin 


282  THE   NERVOUS  DISEASES    OF  CHILDREN. 

is  disintegrated,  and  the  axis  cylinder  either  swollen  or  distorted  ;  but  these 
degenerative  changes  can,  as  a  rule,  be  traced  only  a  short  distance  from  the 
diseased  cord. 

Secondary  changes  follow  upon  the  area  of  inflammation,  and  the  tracts 
will  be  affected  in  an  upward  or  downward  direction,  according  to  the  direc- 
tion in  which  they  transmit  impulses.  Thus,  after  a  transverse  myelitis  the 
lateral  columns  will  degenerate  downward  throughout  their  entire  extent,  but 
an  ascending  degeneration  will  occur  in  the  parts  that  transmit  impulses  in  a 
centripetal  direction.  (Fig.  64.)  Among  those  exhibiting  ascending  degen- 
eration are  the  posterior  columns,  the  cerebellar  tract,  and  the  antero-lateral 
ascending  tract.  The  inflammation  spreads  a  short  distance  upward  and 
downward  by  contiguity,  and  those  parts  which  one  would  suppose  to  be 
subject  to  descending  degeneration  only  may  be  affected  for  a  short  distance 
above  the  lesion ;  but  such  changes  are  of  a  distinctly  inflammatory  charac- 
ter and  altogether  different  from  the  purely  secondary  changes,  which  rarely 
offend  against  physiological  principles.  These  degenerations  are,  as  a  rule, 
developed  very  promptly  after  a  transverse  lesion,  and  often  continue  to  exist 
after  the  initial  inflammation  has  pretty  well  disappeared. 


Whether  fibres  that  have  once  been  seriously  altered, 
or  even  destroyed,  can  ever  regain  their  function  or  can 
grow  anew,  is  a  matter  of  serious  doubt,  and  yet  recovery 
takes  place  in  a  fair  number  of  cases  in  which  absolute  paral- 
ysis, with  signs  due  to  descending  degeneration,  had  existed 
for  a  number  of  months,  or  sometimes  for  a  year  or  more. 
We  must  suppose  in  such  cases  that  some  fibres  were  so 
little  altered  that  when  the  inflammatory  products  were  ab- 
sorbed they  still  retained  the  power  of  conduction,  and  it  is 
more  probable  that  such  fibres  may  be  restored  to  absolute 
health  than  that  entirely  new  fibres  can  be  formed  within  a 
nerve-sheath,  or  that  destroyed  fibres  can  be  replaced  by 
new  ones. 

Differential  Diagnosis. — The  more  or  less  acute  on- 
set, the  often  sudden  loss  of  power,  the  rapid  spread  of  anaes- 
thesia, the  permanency  of  all  these  symptoms,  together  with 
the  retention  of  urine  and  fasces,  and  the  flaccid  and  atro- 
phic symptoms  at  the  level  of  the  injured  part,  together 
with  the  spastic  symptoms  in  the  parts  supplied  from  seg- 
ments below  the  level  of  the  lesion — all  these  symptoms 
will  leave  little  doubt  of  the  diagnosis  of  acute  myelitis.  In 
addition  to  this  the  etiological  factors  in  the  case — *^e  occur- 
rence of  traumatism,  k.  preceding  syphilitic  infectio^  or  pre- 


ACUTE  MYELITIS.  283 

ceding  bone  disease,  or  marked  infectious  influences — will 
help  to  corroborate  the  diagnosis. 

Acute  myelitis  may  resemble  hemorrhage  of  the  cord,  but  in  cases  of  hem- 
orrhage the  onset  is  more  sudden  than  in  cases  of  acute  inflammation,  all  the 
symptoms  being  developed  within  a  very  few  minutes.  There  is,  as  a  rule, 
too,  much  more  pain  than  in  cases  of  myelitis ;  but  hemorrhage  is  frequently 
enough  the  first  stage  of  a  myelitis,  and  if  symptoms  indicating  a  spread  of  dis- 
ease follow  upon  what  is  supposed  to  be  an  initial  spinal  hemorrhage,  it  is  fair 
to  conclude  that  a  myelitis  haemorrhagica  has  followed  upon  the  initial  extrava- 
sation of  blood.  Direct  injury  to  the  spinal  column  may  be  another  factor 
tending  to  corroborate  the  diagnosis  of  hemorrhage. 

A  rapidly  ascending  myelitis  may  suggest  the  acute  ascending  (Landry's) 
paralysis,  but  in  cases  of  myelitis  the  progress  will  be  clearly  from  the  level 
of  the  first  injury,  and  is  not  apt  to  attack  the  parts  in  succession  from  below 
upward,  including  the  trunk,  as  in  cases  of  Landry's  paralysis.  In  ascending 
myelitis,  moreover,  sensation  is  disturbed  from  the  start,  and  all  the  trophic, 
as  well  atrophic,  symptoms  are  much  more  characteristic  of  a  myelitis  than 
they  are  of  Landry's  paralysis.  But  if  the  myelitis  begins  in  the  lumbar  por- 
tion of  the  cord,  and  gradually  spreads  upward,  the  difficulties  of  diagnosis 
may  be  extremely  great.  Landry's  paralysis  is  unusually  rare  in  children, 
whereas  myelitis  is  relatively  frequent. 

The  distinction  between  meningitis  and  myelitis  is  not  of 
great  practical  importance,  for  meningitis  is  rarely  present 
without  some  involvement  of  the  cord,  and  if  the  symptoms 
are  purely  meningeal,  they  are  generally  associated  with 
other  symptoms  pointing  to  a  wide-spread  affection.  A  pri- 
mary spinal  meningitis  is  a  great  rarity,  except  as  a  part  of 
cerebro-spinal  disease  or  after  disease  or  injury  of  the  spi- 
nal column.  The  involvement  of  the  meninges  in  a  given 
case  will  be  indicated  by  considerable  pain  in  loco  morbi, 
and  by  the  presence  of  distinct  neuralgic  pain  along  the 
nerves  emanating  from  the  diseased  portion  of  the  cord. 

The  question  at  times  arises  whether  a  case  is  one  of  myelitis  or  multiple 
neuritis.  In  the  latter  symmetrically  located  pains  are  a  more  prominent  symp- 
tom, trophic  disturbances  are  not  so  marked  as  in  myelitis,  and  the  symptoms 
never  incTuafr'^iaiieaHtnd  rectal  disturbances.  Moreover,  we  never  have  that 
combination  of  p_aralytic  and  spastic  symptoms  which  we  so  frequently  find 
in  cases  of  myelkisS-fci  cases  of  multiptTheuritis  affecting  all  four  extremities 
the  symptoms  in  all  the  extremities  are  entirely  the  same,  whereas  incases  of 
myelitis  they  would  be  of  the  flaccid  order,  say,  in  the  upper  extremity,  and  of 
spastic  order  in  the  lower  extremity. 


284  THE  NERVOUS  DISEASES   OF   CHILDREN. 

A  more  difficult  task  it  is  to  distinguish  between  a  mye- 
litis and  hysterical  paralysis,  and  yet  a  careful  examination 
of  the  patient  should  reveal  important  points  of  diagnosis. 
Thus,  in  hysterical  paralysis,  legs  that  cannot  be  used  in 
standing  or  walking  may  be  moved  freely  in  bed ;  the 
rigidity  is  not  so  marked  as  in  cases  of  myelitis,  and  if  pres- 
ent can  be  more  easily  overcome,  as  a  rule,  than  in  myelitis. 
Bed-sores  are  rarely  present  in  hysteria,  and  anaesthesia,  if 
present,  is  anomalous  in  distribution.  The  reflexes,  too,  are 
not  so  distinctly  exaggerated  in  hysteria  as  they  are  in  cases 
of  myelitis.  If  the  symptoms  should  point  to  a  lumbar  af- 
fection, the  lack  of  atrophy  and  the  persistence  of  the  knee- 
jerks  will  help  to  differentiate  the  hysterical  paralysis  from 
a  spinal  paraplegia.  The  bladder  and  rectal  symptoms  are 
also  not  so  marked  in  hysterical  as  in  myelitic  cases.  The 
very  suddenness  of  the  onset  in  hysterical  cases,  the  fact 
that  the  paraplegia  is  frequently  due  to  a  sudden  fright,  or 
a  deep  emotional  condition,  may  also  point  to  hysteria  rather 
than  to  myelitis.  But  in  all  such  cases  the  fact  that  hysteri- 
cal subjects  may  suffer  from  organic  lesion  should  be  borne 
in  mind. 

PROGNOSIS. — The  prospects  in  cases  of  acute  myelitis 
will  vary  according  to  the  level  affected.  Cervical  myelitis  is 
naturally  a  more  serious  disease  than  myelitis  of  lower  por- 
tions of  the  cord,  for  in  the  former  an  extension  upward  to 
the  i-espiratory  and  cardiac  centres  constitutes  one  of  the 
grave  possibilities  of  the  case.  In  these,  as  well  as  in  dor- 
sal and  lumbar  myelitis,  the  danger  to  life  arises  chiefly 
from  the  complicating  conditions,  particularly  from  bed- 
sores, from  cystitis,  and  pyelo-nephritis.  The  earlier  these 
symptoms  set  in  the  graver  the  prospect  of  the  case ;  but 
not  a  few  of  such  cases  get  well  in  spite  of  all  compli- 
cations, and  if  the  myelitis  can  be  proved  to  be  due  to  spe- 
cific disease,  or  to  some  other  form  of  mild  toxic  infection, 
recovery  is  more  probable  than  in  the  traumatic  cases  of 
myelitis  or  those  in  which  the  etiological  factor  was  en- 
tirely unknown. 

The  prognosis  will  also  vary  according  to  the  intensity 
of  the  affection  and  the  extent  of  cord  involved.  If  all  the 
symptoms   are  developed  rapidly,  then  become  stationary 


ACUTE   MYELITIS. 


285 


and  show  not  the  slightest  sign  of  improvement  for  weeks 
or  months,  the  probability  of  spontaneous  recovery  is  ex- 
tremely slight ;  but  any  improvement  which  sets  in,  either 
in  the  form  of  diminution  of  anaesthesia,  of  the  disappear- 
ance of  bed-sores,  or  of  a  slight  gain  in  motion,  is  a  hopeful 
sign  of  greater  improvement  later  on.  I  have  myself  seen 
complete  recovery  in  cases  of  myelitis  in  which  the  palsy 
was  absolute  for  a  period  of  nearly  six  months,  with 
marked  contractures  and  increase  of  the  reflexes  and  with 
slight  vesical  symptoms,  but  I  cannot  recall  a  single  case 
in  which  complete  recovery  set  in  if  deep  bed-sores  de- 
veloped at  an  early  day,  and  marked  cystitis  appeared  very 
early  in  the  disease,  the  only  exception  to  this  rule  being 
in  cases  of  distinct  specific  myelitis. 

Treatment. — In  the  treatment  of  myelitis  the  follow- 
ing plan  should  be  pursued  if  the  patient  is  seen  during 
the  acute  stage.  An  ice-bag  should  be  applied  to  the 
greater  part  of  the  spinal  column;  counter-irritation  may 
be  used,  but  the  danger  of  trophic  changes  in  the  skin 
should  be  remembered,  and  such  trophic  changes  should 
not  be  encouraged  or  started  up  by  an  excessive  use  of 
counter-irritants.  The  patient  should  be  placed  absolutely 
at  rest,  if  possible  with  some  form  of  extension.  His  bow- 
els should  be  thoroughly  purged,  best  by  the  use  of  calo- 
mel, and  the  bladder  catheterized  by  careful  hands  and 
watched  for  the  first  signs  of  a  cystitis.  (If  cystitis  should 
develop,  no  time  should  be  lost,  even  during  the  acute  stage, 
in  beginning  the  usual  treatment  for  such  conditions.)  All 
these  measures  will  tend,  first,  to  make  the  patient  more  com- 
fortable, and,  secondly,  they  will  surely  lessen  the  danger 
from  complicating  conditions.  The  diet  should  be  of  a  mild, 
non-irritating  kind,  and  the  kidneys  should  be  encouraged 
to  greater  activity.  Under  such  conditions  the  administra- 
tion of  small  doses  of  digitalis  or  of  the  acetate  of  potash 
will  be  quite  in  order.  Ergot  was  recommended  years  ago 
by  Brown-Sequard,  and  may  be  administered  with  the  idea 
of  limiting  the  area  of  inflammation.  I  cannot  say  that  I 
have  ever  seen  any  direct  results  from  ergot,  but  it  seems 
to  do  no  harm,  and  considering  the  seriousness  of  the  dis- 
ease it  may  well  be  tried.    In  cases  in  which  there  is  reason 


286  THE   NERVOUS  DISEASES    OF   CHILDREN. 

to  think  that  there  is  much  inflammatory  exudation,  and 
particularly  in  those  of  specific  origin,  the  administration 
of  the  iodides  is  quite  in  order,  or  of  the  mercurials  and 
iodides  combined  ;  but  if  mercurials  are  exhibited  it  is  use- 
less to  give  them  in  any  other  way  than  by  inunction. 

The  main  objects  are  to  prevent  the  serious  complications 
so  common  in  these  diseases,  and  to  give  the  diseased  or- 
gans a  fair  chance  of  spontaneous  recovery.  It  is  of  the 
utmost  importance,  therefore,  in  cases  of  myelitis,  to  keep 
the  patient  absolutely  clean — a  task  not  so  easy  in  view 
of  the  frequent  dribbling  of  urine  and  of  the  involuntary 
passage  of  fasces.  The  child  should  be  placed  upon  a 
water-bed.  Nurses  should,  invariably,  receive  instructions 
to  keep  the  bed-linen  absolutely  smooth,  and  to  promote 
this  end  it  will  be  best  to  have  the  bed  dusted  very  liberally 
with  some  slightly  aseptic  powder.  In  the  case  of  female 
patients,  pads  should  be  put  in  place  to  catch  the  dribbling 
urine  and  to  prevent  its  soaking  adjacent  parts  ;  in  the 
case  of  boys,  urinals  should  be  used  from  the  start.  It  is 
a  common  practice  with  me  to  order  the  patient's  posi- 
tion in  bed  to  be  changed  at  least  every  hour,  so  that  no 
one  part  is  pressed  upon  for  too  great  a  length  of  time. 
If  in  spite  or  all  these  precautions  bed-sores  should  form, 
these  should  be  treated  according  to  the  best  surgical  prin- 
ciples. -'Latterly  I  have  been  in  the  habit  of  dusting  the 
sores  with  dermatol  or  aristol  and  covering  them  com- 
pletely with  light  antiseptic  dressing,  shielding  the  dress- 
ing as  well  as  may  be  from  contamination  by  urine  or 
fasces.  .  It  is  better  to  change  the  dressing  frequently  than 
to  allow  any  infection  of  the  sores  through  uncleanliness. 
In  hospitals  or  among  the  poorer  classes  less  expensive  sub- 
stances, such  as  bismuth,  may  be  used  ;  and  if  cystitis  has 
been  set  up,  the  bladder  should  be  washed  several  times  a 
week  with  some  weak  antiseptic  solution. 

After  the  symptoms  of  the  acute  stage  have  been  suc- 
cessfully treated,  and  the  patient  has  passed  into  a  more 
or  less  chronic  condition,  the  question  arises  as  to  the 
proper  treatment  of  this  latter  stage  of  the  disease.  I  am 
in  favor  of  making  the  attempt  again  and  again  of  affecting 
the    focus    of   inflammation    by  the    administration    of  the 


ACUTE   MYELITIS.  2%J 

iodides.  In  nine  cases  out  of  ten  this  will  be  unavailing, 
and  yet  there  is  no  good  reason  why  the  effort  should  not 
be  made.  But  the  possibility  of  recovery  should  be  well 
weighed  in  the  scales  as  compared  with  the  gastric  disturb- 
ance which  these  drugs  so  often  excite.  The  nutrition  of 
the  child  must  be  maintained  at  all  odds,  and  it  is  far  better 
to 'abandon  the  iodides  than  to  permanently  impair  the  as- 
similation of  food.  If  the  iodides  cannot  be  given,  inunc- 
tions of  the  oleate  of  mercury  or  of  the  usual  mercurial 
ointment  should  be  substituted  for  the  iodides.  Counter- 
irritation  may  be  attempted,  either  by  the  cautery,  by  blis- 
tering, or  by  mere  cupping ;  but  little  direct  good  is  to  be 
expected  from  these  remedies.  The  child  should  be  kept 
absolutely  at  rest,  and  the  spinal  column  should  be  disturbed 
as  little  as  possible.  Electricity  applied  to  the  spinal  cord 
is  of  very  doubtful  utility,  but  there  is  all  the  more  reason 
for  using  it  in  the  treatment  of  the  paralyzed  parts.  It 
does  excellent  service  here  as  a  form  of  exercise  for  the 
maimed  limbs.  Massage  has  very  much  the  same,  and,  I  be- 
lieve, a  better,  effect  than  electricity.  It  tends,  in  addition, 
to  maintain  the  nutrition  of  the  parts,  and  to  overcome  the 
tendency  to  contractures,  which  so  frequently  give  rise 
to  the  most  disagreeable  symptoms  in  these  cases  ;  but  nei- 
ther electricity  nor  massage  should  be  pushed  if  the  invol- 
untary spasmodic  contractions  become  more  frequent,  as 
they  often  do  in  consequence  of  these  measures.  These 
contractions  are  not  harmful,  but  are  extremely  irritating  to 
the  patient,  and  if  inordinately  increased  may  disturb  his 
rest,  and  thus  interfere  with  the  general  nutrition. 

As  soon  as  the  child  has  sufficiently  recovered,  it  is  of 
the  greatest  importance  to  give  it  all  the  fresh  air  possible, 
and,  if  necessary,  to  provide  it  with  a  wheeled  chair,  so  that 
it  can  be  given  its  regular  outing.  Its  diet  must  be  care- 
fully looked  to,  and  all  unnecessary  excitement  should  be 
avoided.  Tonic  measures  may  be  employed  ;  and  if  the 
child  is  in  the  charge  of  a  competent  person  give  lukewarm 
baths,  followed  by  cool  or  cold  douches  of  the  spine,  and 
let  this  be  done  before  the  massage  is  given.  Iron,  quinine, 
or  arsenic  may  be  administered  for  their  general  tonic  ef- 
fect.    Strychnine  may  be  given  in  very  small  doses  in  those 


288  THE   NERVOUS  DISEASES    OF   CHILDREN. 

cases  in  which  there  is  reason  to  think  that  the  substance 
of  the  cord  has  not  been  absolutely  destroyed,  and  that  the 
function  of  the  diseased  parts  could  be  increased  by  the  use 
of  this  drug.  I  know  that  it  is  an  extremely  popular  drug 
in  these  diseases  with  all  physicians  who  are  not  specialists, 
but  I  have  found  it  to  be  a  double-edged  weapon.  If  it  in- 
creases nerve  conduction  for  a  time,  such  improvement  is 
very  apt  to  be  followed  by  a  further  diminution  of  func- 
tion, and  in  other  cases  again  it  produces  annoying  mus- 
cular contractions,  which  are  as  disagreeable  as  those  that 
result  from  an  excessively  strong  electrical  current.  In 
these  cases,  too,  the  physician  who  watches  his  patient 
carefully,  and  does  not  attempt  to  do  too  much,  will  suc- 
ceed far  better  than  he  who  is  continually  meddlesome  and 
ever  anxious  to  change  treatment. 

INJURIES   OF  THE   SPINAL   CORD. 

The  direct  results  of  jumping,  of  falls,  and  of  injury  due  to  falling  weights 
are,  on  account  of  the  more  delicate  nature  of  the  spinal  vertebra?,  more  seri- 
ous in  childhood  than  in  later  years.  A  fall  downstairs,  or  a  simple  fall  out  of 
bed,  or  a  mild  blow  inflicted  by  another  child,  may  be  a  sufficient  cause  to  start 
the  symptoms  pointing  to  actual  injury  of  the  spinal  cord. 

The  symptoms  of  spinal-cord  injury  may  be  extremely  varied.  I  do 
not  propose  now  to  discuss  those  cases  in  which  traumatism  is  the  remote 
and  questionable  cause  of  a  subacute  or  chronic  form  of  spinal  disease  de- 
veloping months,  or  even  years,  after  an  accident ;  but  I  wish  particularly  to 
direct  attention  to  those  cases  in  which  the  spinal  injury  is  followed  immedi- 
ately, or  within  a  period  of  a  few  weeksjjpy  symptoms  which  point  to  some 
traumatism.  In  the  severest  form  of  injury  to  the  cord  there  is  immediate 
"plararysis  both  of  the  motor  and  sensory  functions.  Inasmuch  as  the  injury 
more  frequently  involves  the  dorsal  and  lumbar  portions,  a  spastic  paraplegia 
with  anaesthesia  of  the  lower  extremities,  with  loss  of  vesical  and  rectal  con- 
trol, constitute  the  chief  symptoms  which  may  be  developed  within  a  few 
minutes  or  within  a  few  hours  after  the  injury. 

If  examined  more  in  detail  we  can  determine  by  the  character  of  the 
symptoms,  first,  the  exact  portion  of  the  cord  involved  by  the  injury,  and  sec- 
ondly, the  amount  of  injury  done  at  any  level.  The  question  of  the  exact 
extent  of  the  injury  up  and  down  the  cord  is  determined  by  the  parts  para- 
lyzed, and  more  particularly  by  the  extent  of  the  anaesthesia.  These  cases  are 
indeed  well  calculated  to  illustrate  the  principles  of  spinal  localization.  In 
cases  of  cervical  lesion  the  upper  as  well  as  the  lower  extremities  are  in- 
volved. In  the  upper  extremities  the  paralysis  is  of  an  atrophic  order,  while 
spastic  paralysis  is  present  in  the  lower  extremities.     The  anaesthesia  in- 


ACUTE   MYELITIS. 


2»9 


volves  both  the  upper  and  lower  extremities  as  well  as  the  trunk,  to  or 
from  a  level  supplied  by  the  nerves  coming  off  from  the  injured  segment. 
In  cases  of  cervical  injury  the  sympathetic  may  be  involved.  In  cases  of  in- 
jury to  the  dorsal  region,  which  are  the  most  frequent,  the  arms  are  not  af- 
fected, the  lower  extremities  are  in  a  condition  of  spastic  paralysis,  the 
bladder  and  rectum  may  be  involved,  and  the  anaesthesia  extends  from  be- 
low upward  to  a  level  corresponding  to  the  segment  or  segments  injured. 
The  band  of  hyperalgesia,  or  the  upper  level  of  the  anaesthesia,  will  cor- 
respond to  the  upper  limit  of  injury.  If  the 
injury  has  been  done  to  the  lumbar  enlarge- 
ment, the  paralysis  of  the  legs  is  of  a  flaccid 
order,  the  reflexes  are  diminished  or  lost,  the 
vesical  and  rectal  reflexes  lost,  and  the  areas 
of  anaesthesia  will  vary  according  to  the  seg- 
ments involved.  Injuries  to  the  cauda  equina 
are  of  particular  interest  because  of  the  pecul- 
iar character  of  the  anaesthesia,  upon  which 
alone  an  accurate  diagnosis  can  be  based.* 

The  exact  study  of  the  symptoms  will 
often  help  us  to  determine  the  upper  as  well 
as  the  lower  limits  of  injury.  Thus  in  one 
case  which  I  have  had  occasion  to  observe, 
there  was  spastic  paralysis  with  slight  dimi- 
nution of  the  knee-jerks,  but  presence  of 
ankle  clonus.  There  was  spasmodic  twitch- 
ing of  the  legs,  loss  of  vesical  and  rectal 
reflexes,  as  well  as  a  tendency  to  bed-sores. 
The  difference  in  the  behavior  between  the 
knee-jerks  and  the  ankle  clonus  proved  con- 
clusively that  the  injury  had  slightly  involved 
the  upper  lumbar  segments,  chiefly  the  lower 
dorsal  region,  but  that  every  part  of  the  cord 
below  the  upper  lumbar  segment  had  escaped 
injury,  or  else  the  ankle  clonus  would  surely 
not  have  been  present.  In  determining  the 
extent  of  the  cross-section  involved,  a  very  important  question,  and  one  which 
helps  us  to  decide  whether  a  complete  crush  has  taken  place  or  not,  we  must 
keep  the  physiology  of  the  cord  in  mind,  and  endeavor  to  make  out  whether 
the  anterior  horns  as  well  as  the  lateral  or  the  posterior  columns  have  been 
involved.  Thus,  in  a  case  in  which  the  lesion  was  in  the  lowTer  dorsal  region, 
and  in  which  there  was  complete  anaesthesia  with  marked  spastic  paralysis,  I 
found  that  the  muscles  of  the  back  showed,  after  a  lapse  of  weeks,  neither  a 
tendency  to  atrophy  nor  to  changes  in  electrical  reactions.  I  argued  from 
this,  and  correctly  too,  as  the  event  proved,  that  the  chief  injury  was  done 
to  the  posterior  and  lateral  portions  of  the  cord,  and  that  the  ventral  portions 
had  entirely  escaped. 

*  See  the  paper  by  Starr  in  American  Journal  of  the  Medical  Sciences,  July,  1892. 


Fig.  68. — Sketch  of  Section  of 
Spine  in  a  Case  of  Fracture 
Dislocation  of  the  Seventh 
Cervical  Vertebra.  (After 
Thorburn. ) 


29O  THE  NERVOUS  DISEASES   OF   CHILDREN. 

Another  aid  to  accurate  diagnosis  of  the  lesion  will  be  found  in  the  sen- 
sitiveness to  pressure  over  the  spinal  column  at  the  seat  of  injury.  The 
parts  that  are  sensitive  should  correspond  to  the  segment  of  the  cord  which 
an  examination  of  the  patient  has  shown  to  be  diseased,  and  before  coming 
to  any  definite  conclusion  the  physician  should  remember  the  relation  of  the 
external  parts  to  the  segments  of  the  cord.  If  these  two  sets  of  facts  do  not 
accurately  correspond,  the  preference  should,  to  my  mind,  be  given  to  the 
seat  of  injury  as  determined  by  the  study  of  the  paralysis  and  the  anaes- 
thesia ;  but  if  the  level  determined  in  this  way  is  not  far  distant  from  the  seat 
of  pain  on  pressure,  both  should  be  included  within  the  area  to  be  operated 
upon. 

The  course  of  the  disease  will  depend  largely  upon  the  region  affected 
and  upon  the  extent  of  injury  done.  Injuries  to  the  cervical  region  are,  on  the 
whole,  more  serious  than  those  to  the  dorsal  and  lumbar  portions  of  the 
spinal  cord.  Injury  to  the  lumbar  spine  is  generally  followed  by  more  serious 
symptoms  than  is  the  case  after  dorsal  injury.  If  the  initial  symptoms  in- 
dicate a  comparatively  slight  lesion  at  any  level,  the  progress  of  the  disease  is 
apt  to  be  more  favorable  than  if  a  complete  or  nearly  complete  crush  of  the 
cord  has  occurred.  If  the  symptoms  show  a  tendency  to  improvement  after 
a  few  weeks,  or  after  a  month  or  more,  the  possibility  of  complete  recovery 
may  be  considered  ;  but  if  they  remain  stationary  for  a  long  period  without 
the  slightest  indication  of  improvement,  actual  recovery  is  rare,  unless  re- 
lieved by  operation. 

The  danger  to  life  is  greater  in  the  cervical  cases,  in  which  the  proximity  to 
the  vital  parts  is  of  much  importance  ;  and  in  severe  cases  of  dorsal  or  lumbar 
injury  the  complicating  conditions,  such  as  cystitis  and  bed-sores,  may  bring 
about  a  rapidly  fatal  issue.  After  an  initial  injury  that  is  relatively  slight,  de- 
generation may  set  in,  which  will  be  characterized  by  the  onset  of  rigidity  and 
contractures,  and  from  the  onset  of  these  symptoms  the  prognosis  as  regards 
complete  recovery  may  become  very  much  graver. 

PATHOLOGY. — The  actual  anatomical  changes  in  traumatic  lesions  of  the 
spinal  cord  may  vary  greatly.  Even  without  injury  to  the  vertebral  column 
hemorrhage  may  occur  from  the  effect  of  the  shock,  and  this  may  be  either 
epidural  or  subdural.  The  probability  of  the  seat  of  the  hemorrhage  will  have 
to  be  argued  from  the  general  character  of  the  symptoms.  In  persons  whose 
arteries  are  fragile,  hemorrhage  is  much  more  likely  than  in  persons  whose 
vascular  system  is  entirely  normal.  Persons  with  syphilitic  disease  will, 
therefore,  be  much  more  liable  to  traumatic  hemorrhage  than  those  not  so 
affected.  If  the  hemorrhage  is  considerable,  whatever  its  location  may  be, 
the  cord  will  suffer  from  compression  and  may  undergo  softening  unless  the 
blood  that  is  exuded  is  rapidly  absorbed.  In  all  cases,  inflammatory  prod- 
ucts, which  may  be  tinged  by  blood,  will  be  found  at  the  seat  of  injury.  At 
times,  in  spite  of  a  sudden  paralysis  developed  immediately  after  an  injury, 
no  tangible  lesion  can  be  discovered  at  the  time  of  operation  or  on  the  post- 
mortem table.  We  must  then  suppose  either  that  the  evidences  of  the  initial 
lesion  have  disappeared,  or  that  the  traumatism  has  resulted  in  functional 
changes,  or  in  such,  at  least,  as  are  beyond  the  discovery  by  our  present 


ACUTE  MYELITIS.  29 1 

methods.  If  examined  months  or  years  after  the  initial  injury,  the  cord  may 
present  nothing  but  the  ordinary  symptoms  of  chronic  myelitis  with  consider- 
able shrinkage  and  wasting  of  the  entire  substance  of  the  cord.  Such  wast- 
ing may  be  at  times  more  marked  in  the  while  columns,  at  other  times  more 
distinct  in  the  gray  matter.  In  addition  to  the  local  changes  found,  the  cord, 
if  examined  carefully,  will  reveal  ascending  and  descending  degenerations  in 
accordance  with  the  intensity  of  the  process  at  any  given  level.  I  have  seen 
several  cases  of  spinal  injury  with  severe  spinal  symptoms  in  which  at  the 
time  of  the  operation  no  tangible  changes  were  found  in  the  cord,  but  the 
cord  was  evidently  compressed  by  inflammatory  exudations  that  had  collected 
between  the  bone  and  the  dura.  Adhesions  also  form  under  these  conditions 
between  the  dura  and  the  surrounding  parts.  The  impairment  of  function  is, 
therefore,  due  to  extra-spinal  conditions.  The  breaking  up  of  such  adhesions 
and  the  removal  of  such  inflammatory  products  are  followed  by  improvement 
in  the  condition  of  the  patient,  if  such  removal  is  effected  within  a  relatively 
short  period  of  time  after  the  accident. 

Treatment. — In  cases  of  serious  injury  to  the  spinal  cord,  absolute 
rest  is  essential.  The  case  may  be  treated  in  every  respect  for  the  first  few 
days  as  though  it  were  a  case  of  non-traumatic  myelitis,  that  is,  by  applica- 
tion of  cold  to  the  spine  and  by  extension.  As  soon  as  the  condition  of  the 
patient  will  permit,  a  careful  examination  should  be  made  in  order  to  de- 
termine the  amount  of  injury  done.  The  details  of  the  treatment  will  not 
vary  from  that  advised  in  cases  of  myelitis.  The  old  habit  of  using  the 
actual  cautery,  of  blistering,  and  the  like,  is  not  to  be  recommended,  for 
little  good  can  follow  it.  As  was  said  in  the  case  of  myelitis,  the  danger  of 
trophic  disturbances  in  the  skin  is  great  enough  without  such  additional  en- 
couragement. The  most  important  question  that  arises  is  whether  anything 
can  be  done  for  the  patient  by  surgical  means.  There  is  still  much  hesita- 
tion on  the  part  of  physicians  and  surgeons  in  this  respect. 

If  the  patient  is  in  good  general  condition,  and  is  able  to  stand  the  shock 
of  the  operation,  the  advisability  of  such  surgical  interference  should  be  con- 
sidered purely  upon  the  merits  of  the  case,  and  if  the  evidence  points  to  the 
fact  that  injured  bone  is  pressing  upon  the  cord,  or  that  severe  hemorrhage 
has  occurred  and  that  there  is  danger  of  permanent  harm  to  the  cord,  the  sur- 
geon should  be  permitted  to  expose  the  injured  region.  This  can  be  done 
with  considerable  impunity  by  competent  surgeons  at  the  present  day,  and, 
as  I  have  said,  should  be  done  as  early  as  practicable  after  the  injury.  The 
cases  in  which  surgical  interference  is  useless  are  those  in  which  all  the 
symptoms  indicate  absolute  crush  of  the  cord,  or  in  which  the  rapid  devel- 
opment of  all  the  symptoms  points  to  a  probably  fatal  issue ;  or  those  in 
which  so  long  a  period  of  time  has  elapsed  since  the  injury  that  there  is  no 
good  reason  to  believe  that  the  conditions  can  be  relieved  by  operation.  I 
have  advised  operations  upon  the  spinal  column  in  four  cases,  two  of  which 
were  in  children  under  the  age  of  fifteen.  The  results  were  satisfactory,  al- 
though neither  one  attained  complete  recovery  ;  but  I  attribute  this  to  the 
fact  that  the  cases  were  sent  to  me  at  too  late  a  period  after  the  accident. 
The  operations  are  not  nearly  so  dangerous  as  those  upon  the  brain,  and 


292  THE  NERVOUS  DISEASES   OF   CHILDREN. 

much  encouragement  may  be  derived  from  the  statistics  of  various  surgeons 
who  have  not  lost  a  single  case  operated  upon  for  serious  spinal  injuries.  If 
for  some  reason  or  other  the  operation  has  not  been  performed  and  the  patient 
is  left  with  a  chronic  spinal  disease,  the  attempt  may  be  made  to  benefit  the 
patient  by  the  administration  of  the  iodides ;  and  the  crippled  condition  of  the 
extremities  may  be  somewhat  improved  by  ordinary  surgical  and  orthopedic 
measures,  such  as  have  been  frequently  referred  to  in  the  discussion  of  mye- 
litis and  other  forms  of  palsy. 


CHAPTER  XVI. 

SYPHILIS   OF   THE   SPINAL  CORD:    SPECIFIC  MYELITIS  AND 
MENINGO-MYELITIS. 

In  the  adult,  syphilis  of  the  spinal  cord  can  now  be  recog- 
nized by  a  definite  combination  of  symptoms.  This  is  due 
to  the  work  of  Erb,  Gowers,  Marie,  Oppenheim,  Siemerling, 
Hoffmann,  the  author,  Nonne,  and  others.  In  the  child 
syphilitic  disease  of  the  spinal  cord  is  not  nearly  so  frequent 
as  in  the  adult,  but  I  do  not  hesitate  to  devote  a  special  sec- 
tion to  this  subject,  both  because  I  think  such  cases  can  be 
easily  overlooked  and  because  they  present  a  number  of 
points  in  differential  diagnosis  which  are  of  the  greatest 
interest  and  which  must  be  carefully  considered  if  grave 
errors  in  diagnosis  are  to  be  avoided. 

Symptoms. — In  former  days  it  was  customary  to  make 
the  diagnosis  of  syphilis  of  the  spinal  cord  if  the  symptoms 
pointing  to  spinal  lesion  were  irregular  and  would  not  fit 
into  any  of  the  ordinary  types  of  spinal  disease.  As  a  mat- 
ter of  fact  the  nature  of  the  morbid  process  underlying 
these  syphilitic  spinal  diseases  is  such  that  irregularities  in 
distribution,  and  in  the  development  of  the  symptoms,  are 
very  apt  to  occur,  and  we  should  be  able  to  recognize  the 
symptoms  of  syphilitic  disease  whether  or  not  the  patient 
reveals,  or  we  can  prove,  previous  syphilitic  infection. 

In  all  but  a  few  of  the  cases  the  onset  of  the  disease  is 
gradual.  By  degrees  the  legs  or  the  arms,  or  both  the 
lower  and  the  upper  extremities,  which  have  shown  some 
weakness,  become  paralyzed.  One  leg,  or  one  arm,  is  at 
times  more  paralyzed  than  the  other.  This  paralysis  may 
be  of  the  atrophic  kind,  but  is  much  more  apt  to  be  of  a 
spastic  order.     It  is  often  associated  with  intense  pain  (par- 


294  THE  NERVOUS  DISEASES   OF  CHILDREN. 

aplegia  dolorosa),  or  with  anaesthesia — the  anaesthesia  and 
paralysis  may  be  crossed  (Brown-Sequard  type).  The  re- 
flexes are  generally  increased,  rarely  absent.  If  the  specific 
process  is  situated  in  the  cervical  segments  there  are  atrophic 
paralysis  with  loss  of  reflexes  in  the  upper  extremities,  spas- 
tic paralysis  with  rigidities  and  contractures  in  the  lower 
extremities.  The  vesical  and  rectal  reflexes  may  be  inter- 
fered with.  If  the  dorsal  or  lumbar  segments  are  involved, 
the  symptoms  will  closely  resemble  those  following  upon 
myelitis  of  the  respective  segments ;  trophic  disturbances 
may  occur ;  bed-sores  may  develop  ;  in  short,  we  may  have 
all  the  symptoms  of  a  wide-spread  spinal  affection.  It  is  evi- 
dent that  if  we  wish  to  distinguish  between  specific  disease 
of  the  cord  and  the  various  forms  of  acute  or  chronic  my- 
elitis we  must  look  for  some  distinct  points  of  differential 
diagnosis. 

Erb,  referring  to  be  sure  to  the  conditions  in  the  adult, 
has  established  a  type  of  spinal-cord  disease  which  he  pro- 
posed to  call  syphilitic  spinal  paralysis.  This  special  type 
bears  the  following  characteristics :  First,  the  usual  symp- 
toms of  spastic  paraplegia,  with  its  peculiar  gait,  carriage, 
and  movements ;  second,  marked  exaggeration  of  the  deep 
reflexes ;  third,  muscular  contractures,  which  are  slight  as 
compared  with  the  exaggeration  of  the  reflexes ;  fourth, 
involvement  of  the  bladder ;  fifth,  a  slight  yet  distinct  dis- 
turbance of  sensation  ;  sixth,  gradual  onset  of  the  disease  ; 
seventh,  a  decided  tendency  to  improvement.  There  is  no 
doubt  that  this  type  of  spinal  disease,  so  well  characterized 
by  Erb,  does  occur.  The  same  series  of  symptoms  has  been 
recognized  by  Rumpf,  and  since  Erb's  publication  cases  of 
this  description  occurring  in  children  have  been  observed 
by  Friedmann  and  by  myself.  But  I  have  taken  some  pains 
to  prove  that  there  are  other  types,  quite  as  frequent  as  this 
one,  and  that  it  is  a  great  mistake  to  hesitate  in  making  the 
diagnosis  of  spinal-cord  syphilis  unless  the  symptoms  of 
Erb's  type  are  present.  It  is  the  author's  conviction  that, 
if  we  wish  to  make  a  positive  diagnosis  of  syphilis  of  the 
spinal  cord,  we  should  pay  attention  to  the  following  points, 
and  not  exclusively  to  those  presented  by  Erb  as  character- 
istic of  the  special  type  he  has  described. 


SYP HILTS   OF  THE  SPINAL    CORD.  295 

First,  the  most  striking  feature  of  syphilis  of  the  spinal 
cord  is  the  unusual  distribution  of  the  disease  over  the 
greater  portion  of  the  cord,  involving,  as  it  often  dues,  the 
cervical  and  dorsal,  as  well  as  the  lumbar  enlargements. 
Second,  the  slight  intensity  of  the  morbid  process  at  one  level 
as  compared  with  the  extensive  area  involved,  as  evidenced 
by  the  preservation  of  some  of  the  functions  of  the  cord 
with  complete  loss  of  others.  Third,  the  rapid  dwindling 
of  some  of  the  symptoms  and  the  very  chronic  persistence 
of  others.  Thus  in  some  of  my  cases  the  anaesthesia  lasted 
but  a  very  short  time,  while  the  paralysis  was  recovered 
from  with  extreme  slowness.  Fourth,  the  very  frequent 
history  of  other  symptoms  pointing  to  specific  disease  in 
the  same  or  distant  parts  of  the  central  nervous  system. 

To  emphasize  these  views  let  me  state  that  I  should  be  inclined  to  suspect 
specific  disease  of  the  cord  if  the  patient  presents  symptoms  of  paralysis, 
whether  they  be  of  the  spastic  or  flaccid  character,  and  whether  the  contract- 
ures be  slight  or  not,  provided  he  furnish  evidence  of  a  morbid  process  affect- 
ing a  very  large  part  of  the  cord,  and  yet  showing  a  relatively  slight  intensity 
at  any  given  level  of  the  cord.  He  may,  for  instance,  exhibit  the  symptoms  of 
extreme  paralysis,  spastic  or  atrophic,  with  partial  or  slight  anaesthesia  of  the 
parts  paralyzed,  with  little  or  no  involvement  of  the  bladder  ;  or,  as  often  hap- 
pens, he  may  present  traces  of  specific  disease  in  other  parts  of  the  central 
nervous  system.  The  chief  difference,  according  to  this,  between  the  ordinary 
forms  of  myelitis  and  the  specific  diseases  of  the  cord  can  be  understood  if 
we  remember  that  the  symptoms  of  an  acute  or  subacute  myelitis  prove  that 
the  entire  cross-section  of  the  cord  is  affected  almost  simultaneously  and  to 
an  equal  degree,  whence  it  follows  that  in  such  cases  severe  paralysis  is  likely 
to  be  associated  with  severe  anaesthesia,  with  marked  contractures,  with  abso- 
lute loss  of  vesical  and  rectal  control,  with  serious  trophic  disturbances,  and 
so  on  ;  whereas,  in  the  cases  of  spinal  syphilis  the  morbid  process  invades  the 
cross-section  of  the  spinal  cord  partially  and  slowly.  We  may,  therefore, 
find  symptoms  which  point  to  a  very  marked  affection  of  one  or  more  of  the 
systems  of  the  spinal  cord  and  to  relative  immunity  from  disease  of  the  gray 
matter  or  other  portions  of  the  cord.  Thus  we  may  have  extreme  paralysis, 
but  only  slight  anaesthesia ;  or  extreme  loss  of  power,  with  relatively  slight 
rigidity,  as  Erb  pointed  out.  Furthermore,  in  the  ordinary  cases  of  myelitis, 
the  symptoms  point  to  a  certain  portion  of  the  spinal  cord  at  which  the  dis- 
ease is  most  intense,  while  in  cases  of  syphilis  of  the  spinal  cord  the  clinical 
symptoms  show  that  the  disease  involves  a  very  large  portion,  if  not  the  en- 
tire spinal  cord,  yet  affects  each  single  segment  but  relatively  little.  A  still 
further  aid  to  differential  diagnosis  is  the  very  frequent  involvement  of  the 
brain,  at  the  same  time  that  the  majority  of  the  symptoms  point  to  disease  of 


296  THE  NERVOUS  DISEASES   OF  CHILDREN. 

the  spinal  cord,  or  if  these  two  sets  of  symptoms  do  not  set  in  simultaneously, 
we  frequently  have  in  a  patient  who  presents  symptoms  of  a  specific  myelitis 
the  history  of  a  preceding  illness  in  which  the  symptoms  were  of  a  cerebral 
rather  than  of  a  spinal  character.  (Unequal  ocular,  and,  above  all,  unequal 
pupillary  symptoms,  are  very  common  symptoms  of  cerebral  syphilis.)  The 
rapid  and  often  unexpected  recovery,  as  well  as  the  relapses,  help  also  to  dis- 
tinguish these  cases  from  the  usual  forms  of  myelitis. 

In  view  of  the  rarity  of  these  diseases,  or  possibly  of  the 
failure  to  recognize  them  when  they  do  occur,  I  wish  to 
give  a  few  typical  cases.  The  first  one  I  take  from  Fried- 
raann's  article  on  relapsing,  probably  specific  spastic,  spinal 
paralysis  in  childhood. 

A  boy,  five  years  of  age  at  the  time  of  examination.  Nine  months  before 
the  birth  of  this  child  the  mother  miscarried  with  twins  at  the  end  of  the  sec- 
ond month  of  pregnancy.  The  birth  of  the  boy  was  entirely  normal,  but 
the  head  was  said  to  have  been  very  large.  Relative  reduction  of  the  size  of 
the  head  in  the  next  few  months.  Four  weeks  after  birth  a  skin  eruption 
appeared  which  covered  the  entire  body ;  it  was  vesicular  at  first ;  later 
on  it  ulcerated,  and  terminated  in  desquamation.  The  child  began  to  ex- 
hibit normal  mental  development,  learned  to  talk  at  the  end  of  one  year, 
and  began  to  teeth  at  the  age  of  six  months.  At  the  age  of  three  months 
there  was  distinct  difficulty  in  moving  the  arms  and  legs.  Three  months 
later  the  right  arm  could  be  moved,  and  at  the  age  of  one  and  a  quarter  years 
all  four  extremities  appeared  to  be  entirely  normal.  In  the  second  year,  the 
child  having  learned  to  walk  well,  paralysis  again  gradually  developed  in  the 
left  arm,  and  disappeared  once  more  after  six  weeks.  From  the  second  to  the 
fourth  year  the  boy  was  healthy,  and  passed  through  measles  and  diphtheria 
without  any  disagreeable  sequelae.  In  the  fourth  year  he  complained  much 
of  headache,  particularly  in  the  occipital  region,  and  gradually  his  gait  became 
weaker  and  weaker,  and  he  began  to  drag  the  right  leg,  frequently  falling  in 
the  attempt  to  walk.  There  was  slight  difficulty  in  micturition  and  the  legs 
were  somewhat  rigid.  In  this  attack  the  arms  were  entirely  free,  and  there 
was  no  history  of  spasms  or  convulsions  at  any  time.  The  patient  was  in 
this  condition  when  examined  by  Friedmann.  The  only  other  points  of  in- 
terest in  the  case  were  the  very  bad  condition  of  the  teeth,  slight  increase  of 
the  reflexes,  and  entirely  normal  sensation. 

More  recently  J.  Hoffmann  has  reported  the  case  of  a 
boy,  who,  at  the  age  of  twelve  years,  developed  a  typical 
spastic  paraplegia,  evidently  due  to  hereditary  syphilis, 
manifest  signs  of  which  appeared  in  the  first  years  of  life. 

The  following  case,  observed  by  myself,  presents  many 
similar  features : 


l/a 


SYPHILIS   OF   THE   SPINAL    CORD.  2GJ 

E.  S ,  a  girl,  aged  six  years,  born  in  this  country,  of  German  parents, 

was  brought  to  my  clinic  in  June,  1893.  The  mother  gives  a  history  of  pro- 
tracted labor,  but  child  was  entirely  normal ;  began  to  cut  teeth  at  six  months, 
to  stand  and  walk  at  the  age  of  one  year,  and  learned  to  talk  well  before  she 
was  two  years  old. 

The  child  had  been  perfectly  well,  with  exception  of  mild  attacks  of 
whooping-cough  and  measles.  At  the  age  of  five  years  the  mother  noticed 
that  the  child  began  to  walk  in  a  peculiarly  stiff  manner,  and  that  its  mental 
development  was  somewhat  retarded.  On  examination  we  noted  spastic  par- 
alytic gait ;  spastic  paraplegia  of  lower  extremities,  more  marked  on  left  side  ; 
left  upper  extremity  slightly  paretic  and  rigid  ;  both  knee-jerks  exaggerated  ; 
riceps  and  wrist  reflexes  lively  on  left  side.  Pupils  unequal ;  left  pupil  dilated 
and  does  not  react  to  light ;  right  pupil  reacts  sluggishly  to  light ;  both  pupils 
react  sluggishly  during  accommodation.     No  sensory  symptoms. 

The  suspicion  of  syphilitic  disease  was  strengthened  by  an  examination  of 
the  mother.  She  has  had  two  miscarriages  ;  three  children  died  in  early  life. 
Five  years  ago  (at  the  age  of  thirty  years)  she  had  left  hemiplegia  ;  no  loss  of 
consciousness  ;  recovered  in  a  few  weeks  ;  has  distinct  "  rheumatic  "  attacks ; 
gets  dizzy  while  washing  her  face  in  the  morning  ;  her  pupils  are  unequal  ; 
no  reaction  to  light  or  during  accommodation  ;  knee-jerks  absent ;  slight 
Romberg  symptom ;  no  bladder  trouble  ;  delayed  sensory  perception  in  the 
lower  extremities. 

Thus  we  have  a  tabic,  if  not  a  purely  syphilitic  affection  in  the  mother,  and 
spinal  syphilis  in  the  child. 


Differential  Diagnosis. — It  seemed  to  me  best  to  in- 
troduce most  of  the  salient  points  of  differential  diagnosis 
in  the  description  of  the  symptoms  of  the  disease,  and  the 
comparison  with  myelitis  was  inevitable.  On  the  distinc- 
tion between  myelitis  of  the  ordinary  types  and  specific 
disease  of  the  spinal  cord,  I  need  say  nothing  more.  Syph- 
ilis of  the  spinal  cord  might  be  confounded  with  a  spastic 
infantile  palsy,  particularly  with  a  spastic  diplegia  and 
paraplegia  resulting  from  meningeal  hemorrhage  occurring 
during  the  period  of  labor ;  but  in  these  latter  cases  the 
trouble  can  be  traced  distinctly  to  the  earliest  period  of 
life,  and  there  is  never  any  history  of  relapses,  and  rarely  of 
any  marked  improvement  followed  by  relapse.  Moreover, 
in  the  typical  cerebral  palsies  the  contractures  are  apt  to 
be  more  extreme,  and  defective  cerebral  development  is 
much  more  frequent  than  in  the  syphilitic  cases  coming  on 
subsequently  to  the  birth  of  the  child.  An  acute  infantile 
cerebral  palsy  might    possibly  simulate    specific   disease, 


298  THE  NERVOUS  DISEASES   OF   CHILDREN: 

were  it  not  for  the  far  greater  frequency  of  the  hemiplegic 
form  of  paralysis  in  these  cases  than  in  the  purely  syphi- 
litic types  of  disease.  Special  difficulties  might,  however, 
arise  in  cases  in  which  the  acquired  infantile  hemiplegia 
would  be  proved  to  be  due  to  syphilitic  disease  of  the  ar- 
teries, and  under  such  conditions  a  combination  of  spinal 
syphilitic  disease  with  cerebral  disease  would  not  be  im- 
possible, although  I  have  not  yet  come  across  a  case  of  this 
description.     Spinal  syphilis  in  a  child  should  not  be  con- 


FiG.  69. — Case  of  Multiple  Cerebrospinal  Syphilis.  Section  through  pons  showing 
gummatous  formation  {g)  in  ventral  portion,  with  considerable  destruction  of  tissue. 
Weigert's  haemotoxylin  stain  ;  low  power.* 

founded    with    hereditary   spastic   paralysis ;    possibly   the 
latter  may  be  developed  on  a  specific  basis. 

Morbid  Anatomy.  —  The  anatomical  changes  in  the 
spinal  cord,  due  to  syphilis,  may  be  quite  as  varied  in  the 
child  as  in  the  adult.  It  is  well  known  that  syphilis  is  apt 
to  cause  disease  of  the  blood-vessels.  Obliterative  endoar- 
teritis,  with  subsequent  softening  of  the  area  supplied  by 
the  diseased  vessel,  is  perhaps  the  best  known  anatomical 
process  directly  attributable  to  syphilis ;  but  a  general  ar- 

*  Figs.  69-71  are  reproduced  by  the  courtesy  of  the  editor  of  the  New  York  Medi- 
cal Journal. 


SYP////./S   OF   THE   SPINAL    CORD. 


299 


teritis  is  quite  as  frequent  as  an  inflammation  of  the  endo- 
thelium alone.  Bruce  has  directed  attention  to  changes  in 
the  adventitia  (nodose  periarteritis];  moreover,  veins  are 
subject  to  syphilitic  changes  quite  as  often  as  the  arteries 
are  (phlebitis  obliterans,  Greiff).  All  these  vascular  changes 
are  much  more  pronounced  in  the  pial  covering  than  in  the 
substance  of  the  spinal  cord. 

The  investigations  of  other  authors,  as  well  as  my  own, 


Fig.  70.— Section  from  Ventral  Surface  of  Medulla  Oblongata  (high  power),  show- 
ing Infiltration  of  Pia  and  Substance  of  Medulla  (a)  and  Typical  Syphilitic  Arteritis  ; 
Marked  Thickening  of,  and  cellular  proliferation  in  Intima  (■/)  ;  Narrowing  of  the 
Lumen  ;  Cellular  Infiltration  of  Adventitia  (i). 


have  shown  that  syphilis  of  the  spinal  cord  is  more  often 
associated  with  a  subacute  or  chronic  meningitis,  or  me- 
ningo-myelitis,  than  with  any  other  process.  The  disease 
starts,  as  a  rule,  in  the  pia,  and  subsequently  invades  the 
spinal  cord.  There  can  be  little  doubt,  however,  that  the 
changes  may  in  some  instances  be  developed  in  the  reverse 
order. 

In  the  gross  specimen  we  find  the  pia  thickened  and 


3oo 


THE   NERVOUS  DISEASES   OF   CHILDREN. 


often  covered  by  a  thick  gelatinous  substance.  On  micro- 
scopical examination  this  thickening  is  seen  to  be  due  to  a 
proliferation  of  all  the  tissues.  The  cells  are  multiplied  in 
number,  the  nuclei  have  increased,  and  the  blood-vessels 
show  the  characteristic  changes  of  specific  arteritis.  The 
walls  of  these  vessels  are  thickened,  all  of  the  coats  partici- 
pating to  an  equal  degree.  The  process  very  often  starts 
in  the  intima,  but  not  invariably  so,  as  was  maintained  some 
years  ago  by  Heubner.     The  pia  is  generally  adherent  to 


FlG.  71.— Section  through  a  Portion  of  Dorsal  Cord  (Ventral  Surface).  Marked 
thickening  of  pia  ;  cellular  infiltration  of  same,  seen  best  in  that  portion  which  pro- 
jects inward  ;  infiltration  of  substance  of  cord. 

the  spinal  cord,  and  the  cellular  infiltration  extends  from  it 
into  the  substance  of  the  spinal  cord.  This  invasion  is  a 
very  gradual  one,  and  may  begin  at  almost  any  point  of  the 
cross-section  of  the  cord  ;  but  the  lateral  columns  are  more 
frequently  involved  than  any  other  region,  and  for  this  rea- 
son the  spastic  symptoms  are,  as  a  rule,  the  first  to  be  de- 
veloped. In  other  cases  in  which  the  morbid  process  in- 
vades the  posterior  columns  the  symptoms  may  resemble 
those  of  tabes  rather  than  those  of  a  spastic  form  of  paral- 


SYPHILIS    OF   THE   SPINAL    CORD. 


30 1 


ysis.     If  the   invasion  occurs  from  the  ventral  surface,  as 
it   rarely    does,    the    symptoms    will    naturally   be    of    an 
atrophic   order   rather  than  of   a  spastic  or  sensory   kind. 
But  wherever  the  first  point  of  attack  may  be,  the   morbid 
process  gradually  works   its  way   inward  from  the  periph- 
ery, and  its   plan   of  attack   is  generally   from  symmetri- 
cally situated  points.      The  cellular  proliferation  and  the 
slight  increase  in  neuroglia  tissue,  as  well  as  the  gradual 
disappearance  of  the  integral  elements  of  the  cord,  give  to 
these  cross-sections,  if  examined  under  the  microscope,  the 
appearance  of  ordinary  myelitis,  and  if  it  were  not  for  the 
characteristic  changes  in  the  blood-vessels  and  for  the  evi- 
dence that  the  process  has  worked  its  way  inward  from  the 
periphery,  and,  indeed,  from  the  pia,  the  anatomical  proof 
of  a  specific  process  would  be  very  difficult  to  establish. 
As  it  is,  there  are  not  a   few  cases  in  which  satisfactory 
proof  cannot  be  given,  and  the  pathologist  is  compelled  to 
rely  upon  the  clinical  evidence  in  the  case  to  prove  the 
syphilitic  nature  of  the  disease.     But  the  wide-spread  char- 
acter of  the  disease,  and  the  very  gradual  destruction  of  the 
different  systems*  of  the  cord,  as  well  as  the  important  part 
played  by  the  pia  and  the  blood-vessels,  leave  little  doubt 
as  regards  the  true  nature  of  the  inflammatory  process,  and 
at  the  same  time  help  us  to  understand  the  peculiar  behavior 
of  the  clinical  symptoms.     The  Figures  69-71  will  give  full 
details  of  the  specific  meningo-myelitis  as  seen  in  one  of  my 
adult  cases. 

The  meningo-myelitis  may  be  associated  with  special 
gummatous  deposits  in  any  part  of  the  cord  or  in  the  brain. 
Thus,  in  one  of  my  cases  a  gumma  in  the  pons  was  asso- 
ciated with  a  wide-spread  specific  meningo-myelitis  at  the 
base  of  the  brain  and  throughout  the  entire  extent  of  the 
cord.  If  such  a  complication  exists  the  child  may  present 
the  symptoms  of  tumor  of  the  brain  or  cord,  together  with 
the  symptoms  of  extensive  meningo-myelitis. 

The  fact  that  spinal  syphilis  has  a  distinct  tendency  to 
improvement  makes  the  prognosis  favorable,  although  this 
tendency  implies  also  a  danger  of  relapses.     But  syphilis  in 

*  Nonne  has  shown  that  Erb's  type  may  be  due  to  a  combined  systemic  degenera- 
tion involving  the  pyramidal,  the  cerebellar  tracts,  and  the  tracts  of  Goll. 


302  THE  NERVOUS  DISEASES   OF   CHILDREN. 

the  spinal  cord  of  the  adult,  as  well  as  of  the  child,  is  amen- 
able to  treatment,  and  the  prognosis  is  distinctly  more  fa- 
vorable than  in  cases  in  which  the  same  symptoms  might 
be  present  and  not  due  to  syphilis.  The  prognosis  is,  for 
instance,  very  much  more  favorable  than  in  cases  of  con- 
genital diplegias  or  paraplegias.  The  possibility  of  recovery 
should  therefore  be  kept  in  mind,  but  the  hope  of  complete 
restoration  can  be  entertained  only  if  the  symptoms  recede 
promptly  upon  anti-syphilitic  treatment.  If  the  symptoms 
do  not  in  any  way  yield  to  treatment,  and  if,  after  a  number 
of  careful  trials  the  condition  remains  practically  the  same, 
the  prognosis  is  as  unfavorable  as  it  would  be  in  any  other 
case  of  myelitis.  The  prognosis  will  also  depend  very 
largely  upon  the  evidence  of  the  amount  of  damage  that 
has  been  done  by  the  specific  process.  If  the  symptoms 
show  that  there  has  been  a  complete  destruction  in  one  or 
more  systems  of  the  spinal  cord  the  possibilities  of  recovery 
are  naturally  less  than  in  cases  in  which  the  symptoms  point 
to  but  a  slight  involvement  of  these  parts.  Yet  whatever 
the  outlook  may  be  at  the  time  the  patient  is  examined,  it  is 
the  physician's  duty  to  warn  the  parents  against  the  possi- 
bility of  relapses  and  to  prepare  the  relatives  for  the  fact 
that  later  attacks  may  involve  more  vital  parts,  and  may, 
therefore,  be  more  dangerous  than  the  one  through  which 
the  child  is  passing  at  the  time. 

Treatment. — Syphilis  of  the  spinal  cord  in  the  child,  as 
well  as  in  the  adult,  calls  for  very  prompt  treatment.  We 
must  depend  upon  the  usual  remedies — the  mercurials  and 
the  iodides.  It  is  wrong  to  depend  upon  either  one  alone, 
for  in  some  cases  the  mercurials  are  more  effective  than  the 
iodides,  and  in  others  the  reverse  is  true.  It  is  my  practice 
invariably  to  begin  treatment  with  one  drug,*  and  to  stop 
mercury  or  the  iodide  if  either  is  not  well  tolerated  or  if 
the  improvement  is  such  that  I  find  it  unnecessary  to  push 
either  drug  to  the  extreme.  In  some  instances  it  is  evident 
that  all  forms  of  specific  treatment  are  of  no  avail.  Mercury 
should  be  given  in  the  form  of  inunctions,  either  of  the  ten 
per  cent,  oleate  of  mercury  or  of  the  unguentum  hydrargyri. 

*  I  agree  with  Lewin  that  it  is  best  not  to  give  mercurials  and  iodides  in  combina- 
tion. 


SYPHILIS   OF  THE  SPINAL   CORD.  303 

According  to  the  age  of  the  child,  one-half  to  one  gramme 
of  the  ointment  may  be  rubbed  in  daily.  If  iodides  are  to  be 
exhibited  begin  giving  small  doses  of  the  saturated  solution 
of  the  iodide  of  sodium,  the  daily  dose  to  be  increased 
slowly.  Thus  I  begin  with  three  or  four  minims  of  this 
solution,  to  be  given  in  milk  three  times  a  day,  and  increase 
the  daily  dose  by  one  minim  until  a  child,  according  to  its 
age,  takes  ten,  fifteen,  or  twenty  minims  of  the  iodide  three 
times  a  day.  As  soon  as  decided  improvement  takes  place 
the  quantity  of  the  mercurial  inunction  given  may  be  re- 
duced and  soon  stopped  altogether ;  but  the  iodides  may  be 
pushed  for  some  time  after  this  improvement  has  been 
noticed.  In  cases  in  which  no  improvement  occurs,  in  spite 
of  the  proper  administration  of  these  drugs,*  it  is  well  to 
stop  both,  for  a  time  at  least;  but  I  would  advise,  under  all 
circumstances,  if  the  diagnosis  is  safely  established,  to  make 
repeated  trials  of  these  drugs  in  the  manner  indicated 
above ;  and  if  a  child  has  passed  through  several  attacks  of 
specific  myelitis,  it  would  be  well  to  subject  it  to  periodic 
treatment  by  the  iodides  in  the  same  manner  that  one 
would  administer  treatment  for  constitutional  syphilis.  In 
addition  to  the  anti-syphilitic  treatment,  the  physician  will 
have  to  prescribe  tonics,  sufficient  exercise,  and  nutritious 
diet,  according  to  the  needs  of  the  patient.  Moreover,  in 
each  attack,  or  as  long  as  the  paralysis  lasts,  it  will  be  neces- 
sary to  use  electricity  and  massage,  as  one  prescribes  them 
in  all  other  cases  of  myelitis,  whatever  the  origin  of  the  my- 
elitis may  have  been. 

*  Oppenheim  advocates  the  use  of  iodipin. 


CHAPTER   XVII. 

DISSEMINATED   SCLEROSIS. 

Disseminated,  insular,  or  multiple  cerebrospinal  scle- 
rosis is  distinctly  a  disease  of  early  life.  The  first  pro- 
nounced symptoms  appear,  generally,  in  the  second  and 
third  decades  of  life,  but  a  few  prodromata  can  be  traced 
back  to  a  much  earlier  period.*  The  disease,  as  described 
by  Charcot,  is  characterized  by  a  very  definite  set  of  symp- 
toms ;  but  variations  from  this  type  are  not  infrequent. 

Symptoms. — The  prodromal  symptoms  of  this  disease 
consist  of  a  weakness  of  the  upper,  and  sometimes  of  the 
lower,  extremities,  of  a  slight  awkwardness  and  trembling 
of  the  fingers,  and  of  slight  subjective  sensory  disturbances 
in  the  arms  and  legs.  The  chief  symptoms  develop  gradu- 
ally, and  are  present  from  the  early  periods  of  the  disease  to 
the  very  end,  but  vary  much  in  intensity  at  different  periods 
of  the  disease.  The  most  characteristic  symptom  is  the 
tremor  which  is  observed  on  voluntary  motion  of  the  af- 
fected parts.  It  is  not  observed  when  the  parts  are  at  rest, 
and  only  when  a  voluntary  effort  is  made  ;  it  is  spoken  of  as 
"  intention  tremor."  The  tremor  consists  of  about  six  to 
seven  oscillations  per  second  (Grasset).     It  becomes  most 

*  Totzke  stated  that  some  of  the  symptoms  were  manifested  in  two  cases  at  birth  ; 
in  one  case  at  the  age  of  five  months,  in  one  at  fourteen  months,  and  in  two  at  the  age 
of  two  years  ;  and  of  thirty-one  cases  which  the  same  author  analyzed,  a  very  fair  pro- 
portion began  between  the  fourth  and  fourteenth  years.  There  is  some  reason  to  doubt 
whether  all  these  cases  were  typical  cases  of  disseminated  sclerosis.  In  my  own  prac- 
tice the  youngest  child  with  distinct  symptoms  of  multiple  sclerosis  was  barely  eight 
years  of  age  at  the  time  of  observation,  and  her  symptoms  did  not  come  on  before  the 
tenth  year.  Marie,  who  reported  three  cases  of  "  sclerose  en  plaques,"  in  infants,  in 
1823,  now  states  that  he  would  not  diagnosticate  the  cases  as  such,  and  believes  the 
"  disease  very  rare  in  children."  Muller,  the  most  recent  author  (1904),  claims  that 
there  is  no  post-mortem  evidence  to  prove  the  existence  of  an  infantile  multiple  scle- 


DISSEMJNA  TED   SCL  ER  OS  IS. 


305 


distinct  if  the  patient  is  asked  to  take  hold  of  any  object, 
to  pass  a  glass  of  water  to  the  lips,  or  to  take  hold  of  a 
pencil,  as  in  drawing  or  writing.  At  first  this  tremor  is  so 
slight  that  it  does  not  interfere  with  the  execution  of  the 
simpler  movements,  but  as  it  increases  in  intensity  it  be- 
comes a  bar  to  the  use  of  the  hands  and  legs,  and  ma)'  be 
an  annoyance  in  walking.  In  attempting  to  put  a  glass  of 
water  to  the  lips  the  water  is  spilt  ("  the  patient's  face  and 
garments  are  inundated  " — Marie),  and  in  writing  the 
tremor  is  so  marked  that  the  pen  tears  the  paper  and  the 
writing  becomes  altogether  indistinct. 

Next  in  importance  to  this  tremor  is  the  difficulty  in 
speech.  The  patient  speaks  deliberately,  slowly,  pronounc- 
ing each  syllable  with  unusual  care  (scanning  speech),  and 
yet  a  distinct  tremulousness  of  the  voice  is  apparent.  The 
consonants  1,  p,  g,  and  r,  are  most  difficult  to  pronounce. 
The  words  in  which  these  letters  occur  become  unintelligi- 
ble at  a  very  early  period  of  the  disease. 

Ocular  symptoms  are  very  common  ;  we  may  have,  at 
times,  an  inequality  of  the  pupils.  (Uhthoff  noted  this  in 
eleven  per  cent,  of  his  cases.)  The  reactions  to  light  and 
during  accommodation  are  diminished,  and  a  myosis  is  gen- 
erally present.  Nystagmus  appears  at  a  very  early  day, 
particularly  if  lateral  movement  of  the  eyes  is  attempted. 
Upward  or  downward  motion  is  unaccompanied  by  this 
phenomenon.  Visual  disturbances  are  not  as  common  as 
in  tabes,  but  do  occur  frequently,  according  to  the  excel- 
lent studies  made  of  this  special  symptom  by  Uhthoff  some 
years  ago.  Clinically  we  find  a  narrowing  of  the  field  of 
vision,  particularly  of  color,  and  a  diminution  of  the  actual 
visual  power.  The  trouble  may  be  unilateral  or  bilateral. 
These  symptoms  are  associated  with  atrophic  discoloration 
of  the  papillae,  which  persists  even  though  an  improvement 
in  the  subjective  symptoms  may  take  place.  The  eye  symp- 
toms are  so  similar  to  those  observed  in  listeria,  that  on 
this  account  alone  the  two  diseases  have  frequently  been 
mistaken  for  one  another. 

A  distinct  tremor  of  the  tongue  and  a  peculiar  vague 
expression  of  the  countenance  are  the  other  head  symptoms 
present  in  this  disease.      Charcot  described  a  special  facies 


306  THE  NERVOUS  DISEASES   OF   CHILDREN. 

of  multiple  sclerosis,  and  refers  to  a  vague  and  uncertain 
look,  the  lips  hanging  down  and  half  open.  On  the  whole  • 
there  is  an  expression  of  mild  stupor  on  the  face ;  this  ex- 
pression is  not  altogether  unnatural,  for  the  mental  condi- 
tion is  in  many  cases  abnormal.  The  memory  is  weak  ; 
there  is  lack  of  attention  on  the  part  of  the  patient,  who 
passes  easily  from  laughing  to  crying,  and  vice  versa.  Many 
of  the  patients  are  emotional  to  an  extreme  degree,  and  this 
is  particularly  true,  according  to  my  own  experience,  of 
those  in  whom  the  disease  begins  very  early  in  life. 

The  paralytic  symptoms  are  of  a  spastic  order.  The 
legs  and  arms  are  moved  awkwardly  at  first,  later  with  dis- 
tinct evidence  of  rigidity.  A  spastic  or  spastic-paretic  gait 
is  present,  and  is  associated  with  those  other  symptoms 
characteristic  of  all  spastic  paralysis  and  of  involvement  of 
the  lateral  columns  of  the  cord,  viz.,  rigidity  of  the  muscles 
and  increase  of  the  deep  reflexes.  All  these  symptoms 
may  for  a  long  time  be  slight,  but  as  the  disease  progresses 
they  become  more  and  more  intense ;  the  spasticity  and 
weakness  grow  worse  until  the  patient  becomes  completely 
bed-ridden  ;  and  the  tremor  increases  in  intensity  and  inter- 
feres with  the  use  of  the  extremities.  Speech  becomes 
almost  unintelligible,  the  memory  grows  weaker  and 
weaker,  until  a  truly  pitiable  condition  is  reached.  Ob- 
jective sensory  disturbances  are  not  uncommon  ;  Freund 
has  observed  them  in  twenty-nine  out  of  thirty-three  cases. 
In  fourteen  there  was  a  mere  transitory  anaesthesia,  while 
in  six  cases  he  states  that  there  was  a  more  or  less  perma- 
nent and  complete  loss  of  sensation.  He  also  refers  to  the 
occurrence  of  hyperassthesia,  and  even  to  the  dissociation 
of  sensation,  but  this  latter  form  of  sensory  disturbances  is 
surely  a  great  rarity.  Fortunately,  or  unfortunately  for 
many,  the  disease  covers  a  long  period  of  years,  during 
which  a  tolerable  condition  of  health  is  maintained.  In 
other  cases,  again,  the  symptoms  described  are  developed 
more  rapidly,  and  a  few  others  are  added  which  are  not  in- 
variably present.  This  is  true  of  the  bulbar  symptoms 
leading  to  difficulties  not  only  of  speech  but  of  deglutition, 
and  occasionally  to  interference  with  the  respiratory  func- 
tions. 


DISSEMINATED   SCLEROSIS. 


307 


Paralysis  of  the  ocular  muscles,  above  all  of  those  sup- 
plied by  the  sixth  and  third  nerves,  are  not  infrequent.  Ves- 
ical trouble  has  been  recorded  by  Erb  and  Oppenheim. 
If  we  add  to  the  above  symptoms  the  complete  paralysis 
resulting  from  apoplectiform  attacks  it  will  be  evident  that 

the  disease  may  become  almost  pro- 
tean in  its  manifestations.  Para- 
doxical contractions  and  impulsive 
laughter  are  frequent  symptoms. 

As    important    as    the    positive 
symptoms    hitherto    recorded    are 
those  of  a  negative  order.    Atrophy 
of  the  muscles,  paralysis  of  the  blad- 
der and  rectum,  and  changes  in  elec- 
trical reaction  are  entirely  wanting. 
Why    such    a   variety    of   symp- 
toms   occurs    in    this    disease    will 
be  understood  easily  enough   when 
we    refer   to    the    morbid    anatomy. 
The    course    of     multiple     cerebro- 
spinal sclerosis  is  slow,  and  though 
invariably  leading  to  a  fatal  issue, 
death  is,   in   the   majority  of  cases, 
the  result  of  some  intercurrent  dis- 
ease. 

Etiology.  — Disseminated  scle- 
rosis comes  on  most  frequently  af- 
ter acute  infectious  diseases,  after 
powerful  emotions,  and  occasionally 
after  severe  injuries.  Oppenheim 
has  shown  that  the  disease  appears 
FV^2%Degenerati0™0,ftlT   after   intoxication  by    metallic  poi- 

Cauda    Equina    in     Multiple  „  J  r 

Sclerosis.    (After  Taylor. )       sons>  while  Marie  believes  it  to  be 
invariably  of  infectious  origin,  com- 
ing on  after  typhoid  fever,  pneumonia,  measles,  scarlatina, 
and  small-pox. 

Heredity  is  supposed  to  play  an  important  role  in  the  eti- 
ology of  insular  sclerosis.  In  the  broader  sense  it  is  true  ; 
for  multiple  sclerosis  may  indeed  affect  children  who  are 
descended  from  a  neurotic  stock  ;  but  it  is  extremely  rare 


3o8 


THE  NERVOUS  DISEASES    OF   CHILDREN. 


to  find  the  disease  in  both  parent  and  child.  The  cases  of 
Pelizaeus,  quoted  approvingly  by  some  authors,  are  not  to 
my  mind  typical  cases  of  multiple  sclerosis ;  they  belong 
rather  to  the  hereditary  form  of  spastic  paralysis.  Krafft 
Ebing,  and  others  mention  refrigeration  as  a  frequent  etio- 
logical factor. 

Pathological  Anatomy. — We  can  infer  from  the  name 
that  the  chief  lesions  in  this  disease  are  irregular  sclerotic 
patches  distributed  throughout  the  greater  part  of  the  cen- 
tral nervous  system.  These  patches  sometimes  occur  in  the 
brain  as  well  as  in  the  spinal  cord  ;  but  there  is  no  rule  de- 
termining their  first  appearance  either  in  one  or  the  other, 
and  there  is,  therefore,  as  little  reason  for  establishing  a 
spinal  form  or  a  cerebral  form  on  anatomical  as  there  is  on 
clinical   grounds.      The  plaques  have,  however,  a  few  fa- 


Fig.  73. — Sections  through  the  Pons,  Medulla,  and  Spinal  Cord,  showing  Sclerotic 

Patches.     (Taylor.) 

vorite  sites ;  the  white  matter  of  the  brain,  the  pons  and 
medulla,  the  lateral  columns  and  particularly  the  posterior 
columns  (Ziegler,  Miiller)  in  the  dorsal  and  lumber  seg- 
ments; but  they  also  appear  in  the  cortex,  in  the  cervical 
region,  and  even  in  the  cauda  equina.  (Fig.  73.)  Charcot 
was  of  the  opinion  that  the  foci  of  disease  were  very  rare 
in  the  cortex  of  the  hemispheres  or  of  the  cerebellum; 
Taylor  claims  they  may  occur  in  these  parts  as  well  as 
in  others.     The  sclerotic  changes  may  also  affect  the  spinal 


DISSEMINA  TE  D   SCL  ER  OS  IS. 


309 


roots  and  various  cranial  nerves.  The  optic  chiasm  is  fre- 
quently diseased.  Whether  the  sclerosis  appears  in  the  cen- 
tral nervous  system  itself,  or  in  the  nerves  emerging  from 
it,  the  character  of  the  changes  is  very  much  the  same,  the 
main  point  being  that  a  few,  and  not  necessarily  the  major- 
ity, of  the  nerve-fibres  are  destroyed. 


These  patches  present 
slightly  elevated,  at  other 
shrunken  and  contracted. 


a  bluish-gray  appearance  and  are  sometimes 
times  the  parts  in  which  they  occur  appear 
In  size  they  vary  from  one  twenty-fifth  to  one 
inch.  They  are,  as  a  rule, 
a  little  harder  than  the  sur- 
rounding tissue,  and  Morris 
calls  especial  attention  to 
the  nicety  with  which  they 
can  generally  be  easily  dif- 
ferentiated from  it  by  the 
naked  eye. 

On  microscopical  ex- 
amination these  patches  are 
found  to  consist  of  a  dense 
fibrous  tissue,  due  undoubt- 
edly to  the  enormous  in- 
crease of  the  neuroglia  fi- 
bres (according  to  Weigert). 
Weigert  insists  that  the  pro- 
liferation of  neuroglia  tissue 
is  more  marked  in  this  dis- 
ease than  in  any  other.  In 
these  sclerotic  portions  the 
myelin  sheaths,  according  to 
the  stage  of  the  disease,  are 
more  or  less  diseased,  but 
the  axis-cylinders  in  the  majority  of  the  cases  remain  normal.  The  entire 
process  may,  therefore,  be  characterized  as  an  interstitial  inflammation,  and 
it  is  owing  to  the  preservation  of  the  axis-cylinder  that  secondary  degenera- 
tion in  multiple  sclerosis  is  a  rare  occurrence,  indeed ;  although  Buss  has 
recorded  a  descending  degeneration  in  the  lumbar  cord  and  an  ascending  de- 
generation of  the  columns  of  Goll,  and  of  the  direct  cerebellar  tract  from  the 
eighth  cervical  segment  into  the  medulla  oblongata,  but  his  observations  are 
surely  exceptional.  The  absence  of  secondary  degeneration  also  accounts 
for  the  frequent  absence  of  contractures.* 

*  The  tremor  of  multiple  sclerosis  has  been  attributed  by  some  to  defective  isola- 
tion (loss  of  sheath)  of  the  axis  cylinders,  on  the  supposition  that  the  nerve  current 
resembles  an  electric  current,  and  the  axis-cylinder  an  electric  wire  ;  but  this  is  rather 
fanciful  pathology. 


Fig.  74. — Vicinity  of  Locus  Cceruleus. 
blood-vessels.     (Taylor.) 


cells  ;  v, 


310  THE  NERVOUS  DISEASES   OF  CHILDREN. 

Considering  the  importance  which  has  been  attached  to  the  acute  infec- 
tious diseases  as  an  etiological  factor  in  multiple  sclerosis,  it  would  be  nat- 
ural to  expect  a  very  early  involvement  of  the  blood-vessels  in  this  disease. 
Such  an  involvement  has  been  found  by  Ribbert,  Greiff,  Cramer,  and  Marie. 
Taylor,  a  more  recent  author  on  this  subject,  whom  I  am  willing  to  follow  in 
this  matter,  because  the  autopsy  in  his  case  was  on  a  young  subject,  proves 
that  in  the  earlier  stages  of  the  disease  there  is  a  distinct  increase  of  the  smaller 
blood-vessels  and  capillaries.  (Fig.  74.)  The  specimens  examined  also  ex- 
hibited small  hemorrhages  and  migrating  white  blood-corpuscles.  In  some 
of  the  vessels  there  was  a  slight  thickening  of  the  walls  and  an  increase  in 
the  number  of  the  nuclei.  The  perivascular  spaces  were  dilated.  The  same 
changes  were  seen  in  a  second  case  of  Taylor's.  In  a  third  case  there  was 
absolutely  no  change  in  the  blood-vessels,  but  in  the  second  and  third  cases 
the  disease  had  lasted  longer,  and  it  is,  therefore,  doubtful  whether  similar 
changes  in  the  blood-vessels  might  not  have  been  present  also  in  these  cases 
in  the  earlier  period  of  the  disease.  Taylor  thinks  that  we  are  not  justified 
in  connecting  these  vascular  changes  with  the  sclerotic  process,  for  the  patches 
are  not  distinctly  related  to  the  diseased  vessels.  Furthermore,  vessels  are 
often  entirely  normal  in  degenerated  areas,  and  in  some  cases  the  disease  of 
the  blood-vessels  is  wholly  wanting,  however  wide-spread  the  lesions  may  be. 

Striimpell  and  M  tiller  regard  the  disease  as  an  endogenous  (congenital) 
affection  (say,  of  the  neuroglia),  stirred  into  being  by  various  exciting  causes. 

Atypical  Forms  and  Differential  Diagnosis. — It 
would  be  an  easy  matter  to  enumerate  a  large  number  of 
atypical  forms  of  multiple  sclerosis,  for  the  clinical  symp- 
toms as  developed  by  Charcot  are  not  distinct  in  many  cases 
which  have,  however,  a  direct  relation  to  this  disease,  repre- 
senting either  early  forms  of  the  same,  or  a  peculiar  local- 
ization of  the  sclerotic  patches.  One  variety  of  multiple 
sclerosis  with  bulbar  symptoms  bears  a  close  resemblance 
to  amyotrophic  lateral  sclerosis.  But  in  children  the  com- 
bination of  symptoms  giving  rise  to  such  difficulty  in  diag- 
nosis is  very  rare.  Then  there  is  the  bulbar,  or,  as  Spitzka 
prefers  to  call  it,  "  the  oblongata  type  "  of  multiple  sclerosis, 
in  which  the  usual  symptoms  are  not  nearly  as  well  devel- 
oped as  are  those  pointing  to  an  involvement  of  the  lowest 
cerebral  centres.  Difficulties  in  deglutition,  in  mastication, 
and  in  articulation  as  well  as  phonation,  are  most  promi- 
nent, and  for  a  long  time  may  obscure  the  usual  symptoms 
of  the  disease. 

A  hemiplegic  form  of  multiple  sclerosis  has  also  been 
observed  in  the  adult,  but  I  have  not  yet  met  with  it  in 


DISSEMINATED  SCLEROSIS.  311 

children ;  nor  have  I  seen  an  apoplectiform  beginning  of 
the  disease  in  children,  as  sometimes  happens  later  in 
life. 

Multiple  sclerosis,  and  forms  closely  allied  to  it  in  children,  should  not  be 
confounded  with  transverse  myelitis.  Acute  and  chronic  forms  of  myelitis  in 
children  are  not  rare.  The  acute  symptoms  and  the  inflammatory  condition 
upon  which  they  depend  disappear  promptly  enough,  but  a  degeneration  is 
set  up  which  leads  to  the  development  of  spastic  and  paralytic  symptoms. 
These  might  be  taken  to  be  symptoms  of  multiple  cerebro-spinal  sclerosis 
unless  the  history  of  the  patient  is  carefully  considered.  The  very  acute 
onset  and  the  involvement  of  the  bladder,  the  persistence  of  sensory  symp- 
toms for  a  shorter  or  a  longer  period  of  time,  will  help  to  differentiate  myelitis 
and  its  secondary  degeneration  from  insular  sclerosis. 

From  congenital  spastic  paraplegia  the  disease  can  be  differentiated  by  a 
consideration  of  the  mode  of  onset,  and  by  the  very  early  appearance  of  the 
congenital  disease,  for  insular  sclerosis  does  not  generally  come  on  until  very 
nearly  the  first  decade  of  life  is  passed.  Moreover,  in  the  congenital  cases  of 
spastic  paraplegia  there  is  frequently  some  cerebral  defect ;  and  the  cardinal 
symptoms  of  sclerosis  are  wanting  in  these  cases.  Westphal  described  a 
pseudo-sclerosis,  a  condition  closely  simulating  that  of  multiple  sclerosis  ;  but 
it  is  not  an  important  disease,  for  to  my  knowledge  it  has  not  been  clearly 
established  in  any  other  instance  excepting  the  one  reported  by  Westphal. 

Cases  of  hereditary  tremor  might  be  mistaken  for  multiple  sclerosis,  and  I 
have  myself  seen  one  patient,  a  girl  aged  twelve,  in  whom  there  was  much  doubt 
as  to  whether  or  not  this  tremor,  which  became  very  much  aggravated  on 
voluntary  motion,  was  the  first  symptom  of  a  multiple  sclerosis  ;  but  having 
observed  the  patient  for  fully  five  years  without  the  addition  of  any  other 
symptoms  referable  to  a  multiple  sclerosis,  I  am  confident  that  the  girl  is  suf- 
fering from  an  hereditary  form  of  tremor,  and  have  since  learned  that  her 
father  and  an  uncle  have  suffered  from  the  same  disease  since  very  early 
childhood. 

The  question  also  arises  in  some  cases  whether  an  insular  sclerosis  or  an 
hysteria  is  present,  but  a  consideration  of  the  symptoms  will  soon  remove  all 
doubts  as  to  the  diagnosis.  Nystagmus,  scanning  speech,  and  intention 
tremor  are  exceedingly  rare  in  hysteria,  while  the  sensory  disturbances  of  hys- 
teria are  too  typical  to  be  mistaken  for  the  slight  subjective  and  objective  dis- 
turbances of  sensation  that  occur  in  multiple  sclerosis. 

In  the  adult  the  chief  point  in  differential  diagnosis  is 
considered  to  be  that  between  multiple  sclerosis  and  paraly- 
sis agitans.  To  this  we  need  pay  comparatively  little  atten- 
tion, since  paralysis  agitans  is  distinctly  a  disease  of  later 
years,  and  is  rare  indeed  before  the  age  of  forty;  but  I  can- 
not forbear  referring  to  the  case  of  a  patient,  whom  I  have 


312 


THE   NERVOUS  DISEASES   OF   CHILDREN. 


observed  many  years,  who,  at  the  age  of  fifteen  years,  pre- 
sented all  the  symptoms  of  genuine  paralysis  agitans.  As 
will  be  seen  from  the  accompanying  cut,  the  position  of  the 
body,  the  expression  of  the  face,  the  position  of  the  hands, 
are  typical  of  paralysis  agitans, 
yet  the  symptoms  have  changed 
in  such  a  way  that  at  the  pres- 
ent time  they  point  to  a  mul- 
tiple sclerosis  rather  than  to  a 
shaking  palsy.     (Fig.  75.) 

The  patient  fell  at  the  age  of  five 
years,  and  had  an  attack  of  convulsions 
with  loss  of  consciousness,  which  was 
followed  by  a  slight  weakness  of  the 
legs,  but  from  this  condition  he  rallied 
rapidly  enough,  and  was  entirely  well, 
exhibiting  good  physical  and  mental  de- 
velopment. He  was  bright  in  school, 
and  perfectly  well  until  the  age  of  fifteen, 
when  he  met  with  another  accident ;  he 
fell  from  a  carriage  and  was  badly  fright- 
ened. 

The  first  symptoms  he  noticed  were 
pains  around  the  left  ankle,  then  a  shak- 
ing of  the  left  leg.  Soon  thereafter  the 
arm  began  to  shake.  Five  months  later 
he  noticed  the  same  symptoms  on  the 
right  side  of  the  body. 

On  my  first  examination  of  the  boy, 
three  years  after  the  accident,  I  found 
him  well  nourished  ;  the  muscles  in  good 
condition,  the  color  of  the  skin  normal, 
but  considerable  vasomotor  disturb- 
ances. He  exhibited  the  mask-like  ex- 
pression of  the  face,  and  his  speech,  as 
well  as  the  rhythmical  tremor  of  the 
hands,  legs,  and  head,  were  exactly  those 
of  a  patient  suffering  from  paralysis  agi- 
tans. 

Soon  another  set   of   symptoms  ap- 
peared, which  proved  to  me  that  this  "  senile  "  disease,  when  occurring  in  a 
young  individual,  was  subject  to  peculiar  modifications,  which  allied  it  much 
more  closely  to  multiple  sclerosis,  a  disease  common  in  earlier  life. 

While  under  observation  he  developed  characteristic  nystagmus,  scanning, 


Fig.  75. — Patient  with  Paralysis  Agi- 
tans, who  also  exhibited  some  Symp- 
toms of  Disseminated  Sclerosis. 


DISSEMINATED  SCLEROSIS.  3  T  3 

and  tremulous  speech.  All  the  reflexes  became  greatly  exaggerated.  The 
typical  tremor  of  paralysis  agitans,  involving  the  head,  lips,  and  tongue,  and 
the  extremities,  remained  unchanged.  He  still  exhibits  the  propulsive  move- 
ment so  characteristic  of  paralysis  agitans.  The  hands  have  the  position  of 
shaking  palsy,  and  the  contractures  are  much  like  those  seen  in  the  senile  form. 
Motion  is  almost  impossible,  and  with  the  symptoms  pointing  in  part  to 
multiple  sclerosis,  and  in  part  to  paralysis  agitans,  he  presents  a  very  unusual 
appearance.* 

Prognosis. — Like  many  other  chronic  disorders  of  the 
central  nervous  system,  multiple  sclerosis  is  not  a  rapidly 
fatal  disease.  It  may  run  a  continuous  course  for  years  with- 
out seriously  endangering  life,  but  any  intercurrent  disease  is 
likely  to  lead  to  a  fatal  termination.  At  all  events,  everyone 
will  acknowledge  that  it  is  rare  to  see  cases  of  multiple  scle- 
rosis that  have  exceeded  the  age  of  forty-five  or  fifty  years, 
and  as  for  those  beginning  very  early  in  life,  the  facts  are 
altogether  too  few  to  warrant  us  in  stating  that  such  pa. 
tients  live  much  beyond  the  middle  period  of  life.  The 
danger  to  life  is  greatest  in  those  cases  in  which  the  ob- 
longata type  predominates.  The  invasion  by  disease  of  the 
vital  centres  might  lead  to  early  death.  The  invasion  is, 
however,  a  very  gradual  one,  and  sudden  death  need  not  be 
looked  for.  In  those  cases  in  which  such  a  termination  is 
threatened,  periods  of  asphyxia,  of  aphonia,  and  other 
symptoms  of  vagus  disturbance  would  occur,  and  would 
give  warning  of  the  terminal  possibilities. 

The  disease  is  absolutely  incurable,  although  remissions 
occur  in  which  all  the  symptoms  excepting  the  tremor  or 
the  nystagmus  disappear,  and  in  some  cases  the  disease  may 
come  to  a  complete  standstill  for  a  period  of  several  years. 
In  this  respect  multiple  sclerosis  is  not  unlike  tabes.  My 
notes  of  the  cases  beginning  in  early  life  would  go  to  show 
that  the  disease  is  even  more  slowly  progressive  than  it  is  in 
the  most  favorable  adult  cases. 

Treatment. — Keeping  in  mind  what  was  said  relative 
to  the  prognosis  of  the  disease  little  can  be  expected  from 
our  attempts  at  treatment.  But  if  we  cannot  cure  the  dis- 
ease we  can  at  least  secure  greater  comfort  for  the  patient. 
As  the   tremor  is  most  marked  when  the   patient    is    ac- 

*  Schultze  has  reported  a  similar  case  in  Virchow's  Archiv,  Bd.  LXVIII. 


314  THE  NERVOUS  DISEASES   OF  CHILDREN. 

tive — and  the  disease  is  exhausting  at  best — it  is  wise  to 
keep  the  patient  in  bed  for  a  prolonged  period  of  time.  The 
rest  cure  I  have  found  as  efficient  in  these  chronic  organic 
diseases  of  the  central  nervous  system  as  I  have  in  the  treat- 
ment of  purely  functional  troubles.  It  is  often  surprising 
to  note  how  much  benefit,  and,  above  all,  how  much  comfort, 
patients  derive  from  a  complete  rest  when  they  have  been 
attempting  to  go  about  from  place  to  place,  often  from  phy- 
sician to  physician,  with  their  weak  and  shaking  limbs. 

With  this  rest  treatment  I  am  in  the  habit  of  com- 
bining mild  hydro -therapeutic  procedures.  Generally  a 
tepid  bath  in  the  morning,  followed  by  lukewarm  and 
successively  colder  douches  down  the  spine,  or  by  the 
use  of  the  drip-sheet.  At  night  it  is  well  to  give  a  tepid 
bath  without  douches,  as  the  former  contributes  much 
to  the  sleep  and  the  general  contentment  of  the  patient. 
The  galvanic  current  may  be  employed  to  advantage. 
Stabile  currents  of  10  to  15  milliamperes  may  be  passed 
down  the  spine  and  through  the  extremities.  The  faradic 
current  should,  however,  be  avoided,  as  it  tends  to  increase 
the  spastic  rigidity  of  the  muscles.  Massage  is  also  in  or- 
der in  these  cases,  if  intelligently  adimnistered.  Spastic 
rigidities  yield  a  little  to  such  manipulations,  and  accord- 
ing to  careful  investigations  of  Mitchell  and  his  son,  the 
general  condition  of  the  patient  is  improved  by  massage. 
If  treatment  is  directed  in  this  way  and  adapted  to  each 
individual  case  it  will  be  far  more  satisfactory  than  send- 
ing the  patient  away  to  various  springs.  The  Frenkel 
method  of  exercises,  that  has  proved  itself  to  be  of  great 
value  in  tabes,  is  certain  to  be  helpful  in  these  cases,  if  in- 
telligently applied. 

Various  drugs  have  been  employed  and  recommended  in 
cases  of  multiple  sclerosis.  Among  these  nitrate  of  silver, 
the  iodides,  and  mercurials  are  the  most  prominent.  I  am 
confident  that  the  first  never  does  any  good  excepting  pos- 
sibly to  allay  pain,  and  the  last  two  will  not  be  needed,  for 
it  is  very  certain  that  multiple  sclerosis  is  rarely  developed 
on  a  syphilitic  basis,  and  there  is  no  indication  for  the  use 
of  resorbents. 


CHAPTER   XVIII. 

COMPRESSION   OF    THE    SPINAL   CORD— POTT'S   PARALYSIS. 

Injury  to  the  cord  may  be  due  to  specific  diseases  in 
the  bone,  to  compression  by  an  aneurism  or  to  growths  orig- 
inating in  the  membranes.  These  conditions  are  very  rare, 
however,  in  children,  and  whenever  the  symptoms  point  to 
compression  of  the  cord,  caries  of  the  bone  may  be  taken  to 
be  the  primary  trouble.  Pott's  disease  of  the  spinal  column 
begins  so  insidiously  that  the  recognition  of  it  and  of  the 
symptoms  resulting  from  compression  are  reached  frequent- 
ly by  exclusion  only.  While  the  disease  may  develop  at 
any  period,  it  is  more  common  in  childhood  and  in  early 
life;  it  is  the  result  of  a  tubercular  or  scrofulous  diathesis; 
and  evidence  is  easily  adduced  in  the  majority  of  cases  of 
the  existence  of  such  diathesis  in  the  same  family  and  in  the 
same  or  in  preceding  generations.  The  slightest  traumat- 
ism may  be  sufficient  to  bring  about  marked  disease  of  the 
bone,  but  evidently  only  in  those  children  who  are  predis- 
posed to  such  disease,  so  that  here  as  elswhere  the  trau- 
matic incident  simply  helps  to  bring  out  the  latent  tendency 
to  disease.  There  is  often  a  period  of  weeks  or  months 
between  the  time  of  injury  and  the  first  symptoms  of  the 
disease.  In  consequence  of  the  caries  of  the  bone,  the  bodies 
of  the  vertebras  become  softened  and  are  displaced,  two  or 
three  of  them,  or  even  more,  forming  a  marked  angular 
curvature.  Such  angular  curvature  may  in  rare  cases  be 
the  result  of  a  septic  process,  in  which  case  an  abscess  may 
have  formed  in  the  bone  and  caused  the  softening.  Gen- 
erally the  bodies  of  the  vertebras  become  diseased  and  the 
intervertebral  substance  undergoes  inflammatory  changes 
as  well.  Vertebral  disease  may  lead  to  retropharyngeal, 
mediastinal  and  psoas  abscesses. 


316  THE  NERVOUS  DISEASES    OF   CHILDREN. 

The  deformity  resulting  from  the  disease  of  the  bone  is 
of  such  a  character  that  a  sharp  angle  is  usually  formed  at 
one  point,  at  which  the  cord  is  generally  much  compressed. 
In  some  instances  the  cord  becomes  quite  thread-like.  It  is 
remarkable  that  the  cord  may  sustain  a  very  large  amount 
of  compression  before  its  functions  are  seriously  interfered 
with,  or  the  symptoms  of  disturbed  function  appear.  This 
accounts  for  the  fact  that  in  many  instances  the  angular 
curvature  may  be  extreme  and  yet  neither  paralysis  nor 
anaesthesia  is  observed  during  life.  Lateral  displacement 
may  occur  without  its  having  any  influence  upon  the  cord. 
Very  naturally  the  spinal  cord  suffers  under  these  condi- 
tions, and  the  roots  emerging  or  entering  through  the  in- 
tervertebral foramina  are  compressed  by  the  thickened 
sheaths  or  by  other  inflammatory  products.  This  accounts 
for  the  frequent  occurrence  of  pain  in  these  cases,  and  for  the 
occurrence  of  paralysis  in  cases  in  which  the  cord  itself 
shows  tolerably  normal  structure. 

The  causes  of  injury  to  the  spinal  cord  in  the  subjects  of 
Pott's  disease  are  twofold  :  first,  the  cord  is  actually  com- 
pressed in  many  cases,  and,  secondly,  it  suffers  from  the  in- 
flammatory products  gathering  between  the  cord  and  the 
bony  parts.  The  latter  cause  is  often  quite  as  active  as  the 
former.  The  changes  in  the  cord  itself  will  vary  very 
largely  according  to  the  degree  of  compression.  The  cord 
is  somewhat  altered,  the  normal  white  having  given  way  to 
a  grayish  or  reddish-gray  tint.  The  cord  is  less  hard  than 
under  normal  conditions,  both  these  changes  being  clearly 
due  to  inflammatory  processes.  It  has  been  found  by 
Schmauss  and  others  that  the  amount  of  inflammation  of 
the  cord  is  not  always  in  keeping  with  the  degree  of  com. 
pression.  Recent  French  authors  have  attached  consider- 
able importance  to  an  oedema  of  the  cord  produced  by 
compression  of  the  veins  as  they  issue  from  the  cord.  The 
oedema  is  subject  to  fluctuations  ;  and  a  diminution  of  the 
same  may  be  responsible  for  an  improvement  in  the  pa- 
ralysis. 

The  symptoms  of  Pott's  paralysis  often  set  in  long  be- 
fore there  is  any  possibility  of  actual  compression.  In  those 
cases  we  must  suppose   that  the  symptoms  are  due  to  a 


COMPRESSION  OF   THE   SPINAL    CORD.  317 

pachymeningitis  constricting  the  spinal  roots  and  blood- 
vessels ;  the  occurrence  of  rapidly  developing  spinal  abscess, 
associated  with  painful  paralysis,  and  the  disappearance  of 
the  paralysis  as  soon  as  the  abscess  is  emptied,  lend  support 
to  this  view.  By  degrees  an  extension  of  the  inflammatory 
process  takes  place  into  the  substance  of  the  cord.  The 
myelitis  as  it  occurs  with  Pott's  disease  shows  a  general  in- 
crease in  the  interstitial  tissue,  with  proliferation  of  the 
various  cells  and  degenerative  changes  in  the  more  impor- 
tant nerve  elements.  Granular  corpuscles,  corpora  amylacea, 
and  globular  masses  of  myelin  are  found  in  sections  that  are 
properly  hardened.  The  gray  matter  may  have  become 
almost  indistinguishable  from  the  white,  and  the  larger 
ganglion  cells  may  have  entirely  disappeared  from  the  for- 
mer. In  addition  to  these  changes  the  usual  ascending  and 
descending  degenerations  will  occur,  as  from  any  other 
focus  of  inflammation  in  the  cord.  The  changes  in  the  pe- 
ripheral nerve-roots  will  be  those  that  would  be  expected 
from  actual  compression  and  inflammation.  The  interstitial 
tissue  will  be  increased,  the  nerve-fibres  themselves  will  be 
wasted,  and  the  axis-cylinders  will  show  the  usual  changes 
of  degeneration. 

Symptoms. — The  chief  symptoms  in  this  disease  are  a 
very  gradual  development  of  a  spastic  paralysis,  generally 
of  the  legs  and  possibly  of  arms  and  legs ;  the  reflexes  are 
increased,  and  radiating  pains  are  present  at  a  very  early 
period  of  the  disease.  The  symptoms  often  resemble  those 
of  a  dorsal  myelitis,  since  Pott's  disease  attacks  the  dorsal 
region  more  frequently  than  any  other.  If  other  regions 
of  the  spine  are  diseased  the  symptoms  will  vary  as  they 
would  in  the  various  forms  of  myelitis,  but  root  symptoms 
will  always  play  a  very  prominent  part.  The  symptoms 
due  to  compression  of  the  root  -  fibres  will  also  help  to 
differentiate  between  a  myelitis  following  Pott's  disease 
and  the  ordinary  traumatic  or  non-traumatic  myelitis.  The 
compression  of  the  sensory  nerve-roots  causes  severe  pains. 
It  may  also  interfere  with  normal  sensation.  (Dissociated 
sensation  has  been  noticed  in  several  of  my  cases) ;  anaes- 
thesia dolorosa  may  be  associated  with  the  paralysis.  Be- 
fore the  condition  of  anaesthesia  is  established  a  transitory 


318  THE  NERVOUS  DISEASES   OF  CHILDREN. 

hyperesthesia  may  have  existed.  The  irritation  of  the 
motor  root-fibres  would  lead  us  to  expect  a  condition  of 
involuntary  contractions  of  the  muscles,  but,  as  a  matter  of 
fact,  these  are  not  so  frequent,  and  probably  because  the 
ventral  gray  matter  of  the  cord,  being  nearest  the  verte- 
bral bodies,  is  affected  at  an  early  day ;  and  since  the  func- 
tion of  this  gray  matter  is  impaired,  irritation  of  the  an- 
terior roots  connected  with  it  will  not  be  able  to  exhibit 
symptoms  of  irritation.  According  to  the  location  of  the 
disease  in  the  bone  the  symptoms  may  for  a  time  remain 
unilateral ;  but,  as  a  rule,  the  disease  spreads  rapidly 
enough  to  bring  about  a  bilateral,  and  often  symmetrical, 
set  of  symptoms  within  a  very  short  period  of  time.  Fur- 
thermore, as  the  disease  progresses  the  paralyzed  parts  may 
become  atrophied,  the  vesical  and  rectal  reflexes  may  be 
impaired,  and  we  then  have  all  the  ordinary  symptoms 
of  myelitis,  even  including  bed-sores  and  the  usual  trophic 
disturbances  of  the  skin. 

The  course  of  the  disease  may  be  inferred  from  the  ra- 
pidity with  which  all  these  symptoms  are  developed.  If 
the  disease  is  of  a  slowly  progressive  character,  the  symp- 
toms will  naturally  be  developed  one  by  one,  whereas  if  the 
bone  disturbance  is  of  a  more  pernicious  and  rapidly  pro- 
gressive character  the  symptoms  of  a  complete  myelitis 
will  be  reached  at  a  very  early  day.* 

Diagnosis. — Paralysis  due  to  Pott's  disease  must  be 
distinguished  from  subacute  or  chronic  myelitis.  This  dis- 
tinction is  not  always  an  easy  one  to  make.  It  is  reached 
most  readily  in  those  cases  in  which  the  course  of  the  dis- 
ease is  a  very  slow  one  and  the  usual  causes  leading  to  a 
transverse  myelitis  are  wanting.  It  is  rendered  probable 
bv  the  cfl£x.i.&tgiice  of  pain  with  the  paralysis,  and  above  all 
things  by  the  discovery  of  tenderness  to  pressure  over  the 
spinous  processes,  /"hese  same  symptoms  might  occur  in 
cases  ot  tumor  5f  the  cord,  but  the  unilateral  development 
of  symptoms  in  the  latter  disease  will  soon  lead  to  a  safe 
differential  diagnosis.  (See  next  chapter.)  The  earlier 
symptoms  of  a  Pott's  paralysis  may  simulate  the  condition 

*In  some  cases,  like  the  one  reported  by  Daxenberger,  the  caries  may  be  latent  for 
years,  before  spinal-cord  symptoms  are  developed. 


COMPRESSION-  OF  THE  SPINAL   CORD.  3X9 

of  spastic  paraplegia,  either  of  the  congenital  order  or  of 
that  form  due  to  specific  disease. 

In  several  cases,  under  the  author's  care,  the  symptoms  of  Pott's  paral- 
ysis were  developed  long  before  any  actual  evidence  of  disease  of  the  verte- 
bra? could  be  elicited.  Whitman  also  states  that  in  a  number  of  cases  the 
paralysis  precedes  the  deformity.  The  diagnosis  must  at  times  be  reached 
by  exclusion  ;  but  the  condition  may  be  suspected  in  cases  of  a  gradually  de- 
veloping spastic  and  painful  paralysis,  in  which  there  is  no  trace  of  preceding 
injury,  and  no  history  of  any  acute  infectious  disease  immediately  preceding 
the  onset  of  the  symptoms. 

The  age  of  the  patient  and  his  general  appearance  will 
also  help  to  establish  a  differential  diagnosis. 

Prognosis. — The  progress  of  the  paralysis  will  depend 
mainly  upon  the  development  of  the  disease  in  the  bone. 
If  the  disease  has  lasted  long  enough  to  produce  complete 
compression  at  one  point,  the  spinal-cord  symptoms  may  per- 
sist during  life,  although  the  bone  disease  be  checked  or  re- 
covered from.  In  other  cases  the  course  of  the  spinal-cord 
disease  may  be  checked,  although  the  disease  of  the  bone 
steadily  progresses.  With  the  subsidence  of  the  inflamma- 
tion the  sensory  symptoms  may  diminish,  but  the  condition 
of  paraplegia  may  continue  years  thereafter  as  a  natural  re- 
sult of  the  changes  that  have  been  set  up  in  the  spinal  cord. 
In  a  number  of  cases,  however,  there  is  a  proportionate  de- 
velopment between  the  disease  of  the  bone  and  the  disease  in 
the  spinal  cord.  In  the  more  severe  cases  the  paralysis  re- 
mains unaltered,  or  is  steadily  progressive,  and  all  those 
complications  arise,  such  as  bed-sores,  cystitis,  and  the  like, 
which  help  to  bring  about  a  fatal  issue  in  many  cases  of  my- 
elitis. In  the  milder  cases  recovery  is  to  be  expected,  and 
many  a  child  who  has  not  been  able  to  walk  for  years  during 
the  active  period  of  caries  has  attained  a  tolerably  normal 
gait  in  later  life.  On  the  whole,  the  motor  symptoms  are 
much  more  persistent  than  the  sensory.  The  prognosis  is 
much  more  grave  in  the  cases  of  cervical  Pott's  disease  than 
in  those  occurring  in  the  lower  vertebral  regions,  for  rea- 
sons which  can  be  readily  understood. 

Treatment.— In   the  treatment  of   paralysis   resulting 
from  Pott's  disease  surgical  methods  should  be  resorted  to 


320  THE  NERVOUS  DISEASES   OF   CHILDREN. 

at  the  very  beginning.  If  the  spinal  cord  is  to  recover,  the 
tubercular  process  in  the  spinal  column  must  be  checked 
first  of  all.  The  old  method  of  absolute  rest  should  be  em- 
ployed, and  tonics,  such  as  cod-liver  oil  and  iron,  should  be 
given.  I  do  not  wish  to  enter  into  the  discussion  as  to  which 
of  these  measures,  the  rest  or  the  tonics,  is  more  impor- 
tant. Some  will  be  cured  by  the  one,  and  some  by  the  other, 
method,  but  I  have  not  the  slightest  doubt  that  if  all  these 
measures  are  employed  conjointly  the  result  will  be  more 
satisfactory.  In  addition  to  rest,  absolute  immobility  should 
be  secured,  either  by  forcible  extension  in  bed,  or  by  the 
application  of  a  plaster-of- Paris  jacket.  The  plaster-of- 
Paris  jacket  is  not  so  useful  during  the  period  of  rest  as  it  is 
during  the  period  of  partial  recovery,  in  which  it  is  desira- 
ble for  many  reasons  that  the  child  should  be  taken  out  of 
bed  and  enabled  to  walk  in  the  open  air.  I  am  very  certain 
that  the  application  of  counter-irritants,  which  is  still  recom- 
mended by  many,  is  of  no  use,  and  simply  helps  to  increase 
the  tortures  that  these  children  suffer.  Of  late  years  active 
surgical  interference  has  been  suggested  in  these  cases  by 
Macewen,  Horsley,  and  others.  Thorburn  and  Chipault's 
statistics  show  a  mortality  of  forty-three  per  cent.  The  sur- 
geon's skill  should,  at  all  events,  be  restricted  to  those  cases  in 
which  the  clinical  symptoms  prove  that  there  is  evidence  of 
compression  at  anyone  special  point  by  inflammatory  prod- 
ucts or  by  displaced  bone.  If  the  child  is  strong  enough  to 
tolerate  such  surgical  procedures,  the  removal  of  these 
products  of  inflammation  may  be  attempted  ;  but  it  is  well 
to  caution  the  physician  who  may  be  eager  to  try  new  sur- 
gical procedures  that  children  are  less  tolerant  than  adults 
of  spinal  and  cerebral  operations.  Callot's  redressement 
forcee  cannot  be  endorsed. 

The  orthopedic  surgeon,  by  well  planned  tenotomies,  may 
enable  the  child  to  use  its  feet  to  greater  advantage.  His 
treatment  will  be  supported  in  every  case  by  the  discreet 
use  of  electricity  and  massage.  The  ordinary  blood  and 
nerve  tonics  will  help  very  much  more  than  the  iodides 
or  mercurials,  but  these  should  be  given  in  the  earliest 
stages  of  the  trouble.  I  have  seen  favorable  results  from 
mercurials  in  cases  in  which  the  process  was  assumed  to  be 
gummatous  and  not  tubercular. 


CHAPTER  XIX. 

TUMORS   OF   THE   SPINAL   CORD   AND  ITS   MENINGES. 

The  study  of  tumors  of  the  spinal  cord  was  at  one  time 
considered  to  be  of  small  practical  value  on  account  of  the 
rarity  of  the  affection  ;  but  far  greater  interest  attaches 
to  it  now  from  the  fact  that  the  removal  of  these  tumors 
has  been  rendered  possible  by  the  recent  advances  in  sur- 
gery. About  fifteen  years  ago  Mills  and  Lloyd  collected 
and  analyzed  fifty  cases  of  cord  tumors ;  fourteen  per  cent, 
of  these  were  under  twenty  years  of  age.  Bruns,  Oppen- 
heim,  Schlesinger,  and  others  speak  of  the  relative  fre- 
quency of  occurrence  of  these  tumors  in  the  early  years  of 
life. 

Causes. — The  actual  cause  of  tumors  of  the  spinal  cord  can  be  deter- 
mined as  little  as  in  the  case  of  cerebral  tumors.  They  are  rarely  primary, 
and  are  commonly  due  to  some  constitutional  diathesis,  such  as  tuberculosis 
or  syphilis,  or  are  the  metastatic  products  of  malignant  tumors  in  other  organs 
of  the  body.  The  tendency  to  proliferation  of  nerve-tissue  is  not  so  marked 
in  the  spinal  cord  as  in  the  brain  of  the  child.  Gliomata  originate  in  the  vicinity 
of  the  central  canal,  and  here  are  of  special  interest,  as  they  often  break  down 
and  lead  to  the  formation  of  cavities,  and  hence  have  given  rise  to  the  discus- 
sion whether  these  cavities  are  invariably  the  result  of  such  disorganization 
of  a  neoplasm,  or  whether  the  condition  of  syringo-myelia  may  be  the  result 
of  congenital  enlargement  of  the  central  canal.  Small  hemorrhages  and  em- 
bolism, though  much  rarer  than  in  the  brain,  may  give  rise  to  cystic  forma- 
tions, but  among  cystic  growths  those  due  to  parasitic  infection,  particularly 
to  the  echinococcus,  are  the  most  common.  As  in  the  case  of  brain  tumor, 
accidental  injury  has  been  declared  responsible  by  many  for  the  occurrence  of 
spinal  tumors.  It  is  doubtful,  however,  whether  traumatism  plays  any  further 
role  than  that  either  of  eliciting  a  latent  tendency  to  such  disease,  or  of 
merely  calling  attention  to  the  symptoms  of  the  disease  which  may  have 
antedated  the  injury. 

Symptoms. — The  symptoms  of  tumor  of  the  cord  might 
be    deduced   from   a   consideration   of  the  disturbance  of 


322  THE   NERVOUS  DISEASES   OF   CHILDREN. 

function  due  to  a  very  gradual  invasion  of  the  cord.      Thus 
in  many  cases  the  symptoms  are  strictly  unilateral  for  a 
time,  and  become  bilateral  with  the  extension  of  the  disease 
from  one  part  of  the  cord  to  the  other.     The  symptoms  will 
naturally  vary  also  according  to  the  seat  of  the  disease,  ac- 
cording to  the  more  or  less  rapid  destruction  of  the  cord, 
and   according   to   the    development   of   the  tumor  in  the 
meninges  or  in  the  spinal  cord  itself.     It  is  of  the  greatest 
importance  to  distinguish  between  extra-medullary  and  me- 
dullary tumors.     Extra-medullary  tumors  are  characterized 
by  the  early  appearance  of  root  symptoms,  more  persistent 
pain  in  the  back,  and  by  sensory  disturbances  which  point 
to  compression  of  the  posterior  root-fibres ;    by  paralysis 
and  atrophy  if  the  growth  is  on  the  ventral  surface.     These 
distinctions  will  not  hold  good  if,  as  in  a  case  of  my  own, 
the  intra-medullary  tumor  starts  near  the  entrance  of  the 
posterior    root-fibres  and  actually  compresses    these.      In 
some  cases,  however,  the  disproportion  between  the    sen- 
sory symptoms   and  the  paralysis  will  direct  attention  to 
the  seat  of  the  tumor  outside  of  the  spinal  cord,  particu- 
larly if  all  symptoms  pointing  to  involvement  of  the    cen- 
tral gray  matter  are  absent.      The  focal  symptoms  in  ex- 
tra medullary,  as   well   as   in  intra-medullary  tumors  will 
vary  greatly  according  to  the  seat  of  the  tumor.     There 
may    be   but  slight  sensory  disturbances   in  a  single  area 
or   in    one    limb;    the   sensory    disturbances    may    be  uni- 
lateral, and  these  may  or  may  not  be  associated  with  par- 
tial paralysis,  either  unilateral  or  bilateral ;  or  there  may  be 
crossed  sensory  and  motor  symptoms.     Any  set  of  spinal, 
and  more  particularly  hemi-spinal  symptoms,  whether  of  a 
motor,  sensory,  or  vasomotor  order,  if  they  be  slowly  pro- 
gressive, are  suspicious  of  tumor.     There  will  be  additional 
reason  to  suspect  tumor  if  there  is  evidence  of  a  distinct 
constitutional   diathesis.      All    the    symptoms    of    various 
spinal-cord  affections  may  be  simulated  by  tumor,  accord- 
ing  to   the   part   of   the   spinal  cord  affected,  and  in  this 
respect   the    symptomatology  of  tumors  may   be  quite  as 
varied  as  that  of  syphilis  of  the  spinal  cord. 

If  we  endeavor  to  analyze  the  symptoms  in  detail,  we 
find  that  disturbances  of  sensation  are  by  far  the  most  com- 


TUMORS  OF  THE  SPINAL  CORD  AND  ITS  MENINGES.      323 

mon.  If  the  tumor  is  in  the  cervical  region,  the  pain  radi- 
ates into  the  upper  extremities,  and  generally  into  definite 
parts  of  the  brachial  plexus  ;  if  in  the  dorsal  region,  the 
pain  is  relegated  to  the  region  of  the  ribs,  the  thorax,  or  the 
abdomen  ;  if  in  the  lumbar  region,  the  pain  is  described  as 
limited  to  the  various  parts  of  the  lumbar  or  sacral  plexus. 
Whatever  the  level  of  the  lesion  may  be,  the  sensory  dis- 
turbances may  be  unilateral  for  a  time  and  gradually  be- 
come bilateral.  Vasomotor  disturbances  occur,  similar  to 
those  in  peripheral  neuritis  ;  the  skin  becomes  glossy,  livid, 
and  painful.  With  these  symptoms  muscular  wasting  and 
paralysis  will  soon  be  associated.  As  the  disease  progresses 
the  hyperalgesia  or  the  paresthesia  gives  way  to  complete 
loss  of  sensation,  the  paralysis  becomes  complete,  and  the 
vasomotor  disturbances  increase,  and  serious  trophic  dis- 
turbances may  set  in.  The  sensory  changes  may  vary  in 
still  another  respect.  Since  the  tumor  need  not  invade  all 
sensory  fibres  at  once,  some  may  remain  entirely  intact, 
while  the  function  of  others  becomes  seriously  impaired. 
Thus  dissociated  sensation  may  be  present  in  some  cases, 
but  is  rarely  as  distinctly  developed  as  in  the  case  of  syringo- 
myelia, which,  as  we  have  seen  above,  may  or  may  not  be 
connected  with  the  formation  of  a  central  neoplasm.  Ac- 
cording to  well-known  physiological  laws,  and  to  the  old- 
time  teachings  of  Brown-Sequard,  one  would  expect  in 
many  of  these  cases  of  tumor  to  find  distinct  symptoms  of 
unilateral  lesion  of  the  cord,  namely,  paralysis  in  one  mem- 
ber or  in  one-half  of  the  body,  with  loss  of  sensation  in  the 
opposite  half ;  but  this,  curiously  enough,  is  not  often  the 
case. 

The  paralysis  will  be  developed  in  accordance  with  the 
ordinary  laws  relating  to  spinal-cord  affections.  The  mus- 
cles represented  in  the  segment  or  segments  involved  by 
the  neoplasm  (see  tables  in  Chapter  I.)  will  not  only  be 
paralyzed,  but  will  also  undergo  atrophy,  while  the  mus- 
cles represented  in  lower  segments  will  show  spastic  forms 
of  paralysis  without  any  atrophy.  The  changes  in  electri- 
cal reactions  will  also  vary  in  accordance  with  these  con- 
ditions, the  only  muscles  exhibiting  distinct  reaction  of 
degeneration  being  those  governed  by  cells  in  the  affected 


324  THE   NERVOUS  DISEASES   OF   CHILDREN. 

segments.  Spasmodic  contractions,  the  ordinary  rigidities 
and  contractures,  will  be  observed  in  these  cases  under  the 
same  conditions  as  in  cases  of  myelitis.  All  the  symptoms 
will  be  largely  influenced  by  the  fact  that  an  active  my- 
elitic process  is  started  up  sooner  or  later  in  the  vicinity  o£ 
most  of  the  tumors.  This  is  particularly  true  of  tubercular 
and  gummatous  deposits,  whereas  other  forms  of  tumor  may 
exist  for  some  period  of  time  without  seriously  affecting  the 
tissue  in  their  vicinity. 

Paralysis  of  the  sphincters  of  the  bladder  and  rectum 
will  occur  in  cases  of  tumor  as  in  cases  of  myelitis.  In  the 
case  of  tumor  in  the  lumbar  region,  absolute  paralysis  of 
the  sphincters  and  retention  are  the  natural  results,  while 
if  the  tumor  is  above  this  level  incontinence  will  be  the 
more  frequent  condition  ;  but  in  the  later  stages  retention 
with  overflow  is  the  rule.  The  conditions  of  the  sphincter 
ani  will  vary  in  very  much  the  same  way.  Tumors  of  the 
cauda  equina  deserve  especial  consideration.  In  addition 
to  the  general  symptoms,  pains  radiating  into  the  sacrum, 
the  perineum,  the  anus,  the  bladder,  and  into  the  distribution 
of  one  or  both  sciatic  nerves  will  be  present.  In  one  of  my 
own  cases,  bladder  and  rectal  symptoms  were  absent  for  a 
long  time.  The  gait  may  be  only  slightly  defective  for 
some  weeks ;  with  the  growth  of  the  neoplasm  the  paralysis 
of  the  legs  will  become  more  and  more  evident.  In  two 
patients  whose  cases  were  reported  by  the  author,  pressure 
to  one  side  of  the  median  line  had  produced  the  precise 
sensory  disturbances  of  which  they  had  complained. 

It  is  well  to  remember  that  a  very  large  number  of  so-called  "  spinal  cord 
tumors  "  start  from  the  vertebral  bodies ;  many  others  from  the  meninges, 
and  that  the  spinal  symptoms  are  in  reality  compression-symptoms,  and  not 
due  to  an  invasion  of  the  cord.  Not  infrequently  in  children,  as  I  had  occasion 
recently  to  see  in  a  lad  of  fourteen  years,  the  spinal  tumors  are  so  clearly 
metastatic  products  (sarcomata)  that  operative  interference  would  be  of  no 
avail.  In  some  instances  of  tuberculosis,  spinal  symptoms  may  appear  to  be 
primary;  but  the  autopsy  will  reveal  the  more  probable  focus  of  infection.  In 
a  case  of  solitary  tubercle  of  the  cord  between  the  sixth  and  seventh  cervical 
segments  I  found  in  addition  that  the  cord  between  the  seventh  cervical  and 
the  fourth  dorsal  segments  was  a  diffluent  mass.  There  were  also  minute 
tubercular  deposits  discovered  in  both  lungs,  and  in  both  apices  there  were 
incipient  cavernous  spaces. 


TUMORS  OF  THE  SPINAL  CORD  AND  ITS  MENINGES.     325 

Pathology.— The   morbid   anatomy  of  tumors  of   the 
cord  differs  very  little   from  that  of  tumors  of  the  brain. 
The  central  nervous  system  is  in  this  respect  a  unit,  and  the 
same  growths  which  are  apt  to  occur  in  the  brain  occur, 
though  with  lesser  frequency,  in  the  spinal  cord.     In  the 
cases  of  Mills  and  Lloyd,  only  four  were  of  a  tubercular 
character,  while  twelve  were  either  sarcomata  or  gliomata. 
Parasitic  growths  occurred  in  three  cases  of  the  fifty,  syph- 
ilitic growths  in  five,  and  the  remainder  were  distributed 
among  various  kinds  of  neoplasms,  such  as  myxoma,  fibroma, 
carcinoma,  and  psammoma.    Neuromata  of  the  cauda  equina 
must  be  included   in    the   list.     The    largest    collection   of 
cases  of  solitary  tubercle  of  the  spinal  cord  has  been  made 
and    carefully   analyzed   by   Dr.    Herter.      His  experience 
proves  that  the  disease  is  one  of  early  life,  twenty  of  the 
twenty-six  cases    occurring  before  the   age  of  thirty-five. 
A  few  authors  have  reported  cases  of  tubercle  which  they 
supposed  to  be  primary ;  but,  as  in  the  case  reported  by 
myself,  although  it  was  difficult  to  prove  any  other  focus  of 
tubercular  disease  during  life,  such  focus  of  disease  evi- 
dently precedes  the  spinal  disease  in  every  instance.     Spe- 
cific infiltration  of  the  spinal  cord  is,  on  the  whole,  more  fre- 
quent than  the  same  infiltration  of  the  brain.     To  be  sure, 
relatively  few  cases  of  this  description  are  on  record,  but  I 
am  certain  that  more  careful  examination  will  hereafter  re- 
veal a  larger  number  of  gummatous  deposits,  if  not  gum- 
matous growths,  in  different  parts  of  the  cord,  associated 
with  the  specific  meningo-myelitis,  which  is,  as  has  been 
stated  in  another  chapter,  the  most  frequent  form  of  syphi- 
litic disease  of  the  cord.     Tumors  of  the  cord  may  occa- 
sionally erode  the  surrounding  bony  parts  and  may  work 
their  way  to  the  surface,  but  this  is  very  much  rarer  indeed 
than  a  similar  invasion  of  the  skull  in  cases  of  brain  tumor. 
In  a  case  of  my  own  the  tumor  (though  a  small  one)  "pre- 
sented "  though  an  intravertebral  foramen. 

Diagnosis. — Tumors  of  the  spinal  cord  are  to  be  differentiated  from 
hemorrhage  into  the  cord,  from  spinal  caries  with  Pott's  paralysis,  from 
pachymeningitis  cervicalis,  transverse  myelitis,  syrmgo-myelia,  neuritis,  and 
from  injuries  of  the  cord. 

In  the  case  of  hemorrhage  the  paralysis  and  other  symptoms  resulting 


326  THE  NERVOUS  DISEASES    OF   CHILDREN. 

therefrom  appear  with  great  suddeness.  The  condition  is  one  of  extreme 
rarity,  and  in  many  of  the  cases  leads  to  complete  paralysis,  with  all  its  com- 
plications, and  to  death  in  a  relatively  short  period  of  time.  A  confusion  can, 
therefore,  exist  only  in  the  first  few  days  of  the  disease,  and  under  such  cir- 
cumstances the  suddenness  of  the  onset  would  argue  strongly  in  favor  of 
hemorrhage. 

Spinal  caries,  with  the  resulting  paralysis,  may  occasionally  present  symp- 
toms similar  to  those  of  spinal  tumor.  In  both,  pressure  symptoms  are  pres- 
ent, but  in  the  case  of  caries  of  the  bone  the  symptoms  are  not  nearly  so 
intense,  as  a  rule,  as  in  those  due  to  the  presence  of  neoplasm.  The  chief 
reliance  in  differential  diagnosis  must,  however,  be  placed  upon  the  detection 
of  bone  trouble  in  the  case  of  spinal  caries,  although,  as  we  have  seen,  such 
evidence  does  not  always  precede  the  onset  of  pressure  symptoms.  The 
more  rapid  course  of  the  symptoms  in  spinal  tumor,  and  the  negative  results 
of  treatment,  will  also  help  to  differentiate  the  two  conditions.  In  caries  of 
the  vertebrae,  symptoms  pointing  to  the  ventral  (anterior)  surface  are  a  little 
more  frequent  than  in  tumor. 

As  between  pachymengitis  cervicalis  and  tumor  of  the  cervical  region,  there 
are  but  few  points  of  differential  diagnosis,  and  the  diagnosis  will  have  to 
rest  very  largely  upon  the  excessive  rigidity,  the  very  typical  distribution  of 
the  paralysis  in  the  hand,  and  the  more  acute  pain  in  cases  of  pachymenin- 
gitis than  in  those  of  tumor.  In  tumor,  moreover,  the  symptoms  are  more 
apt  to  be  unilateral  for  a  time,  while  those  of  pachymeningitis  are,  as  a  rule, 
bilateral  and  symmetrical. 

Some  of  these  same  points  of  differential  diagnosis  will  help  us  also  to 
distinguish  between  transverse  myelitis  and  tumor  of  the  cord,  the  chief 
points  of  difference  being  these :  In  myelitis  the  onset  is  much  more  rapid 
than  in  tumor,  there  is  less  pain,  and  the  entire  cross-section  of  the  cord 
shows  symptoms  of  involvement  very  much  more  promptly  in  myelitis  than 
in  the  majority  of  cases  of  neoplasm.  A  doubt  as  to  which  of  these  two 
conditions  is  present  can  exist  in  the  first  few  days  or  weeks  only  ;  after  this 
the  general  course  of  the  symptoms  will  determine  the  character  of  the  morbid 
process. 

Syringo-myelia  is  closely  related  to  tumor  of  the  cord  ;  there  is  doubt,  in 
fact,  whether  some,  if  not  many,  of  the  cases  of  syringo-myelia  do  not  repre- 
sent primary  neoplasm  of  the  cord,  with  secondary  enlargement  of  the  central 
canal  and  a  breaking  down  of  the  spinal  tissues.  But  the  gliosis  which  may 
or  may  not  be  the  starting-point  of  a  syringo-myelia,  takes  an  extremely 
chronic  course,  and  it  is,  therefore,  by  this  fact  alone,  as  well  as  by  others, 
that  the  two  diseases  can  be  sufficiently  distinguished  from  one  another.  In 
syringo-myelia  the  symptoms  are  often  strictly  bilateral,  or  symmetrical,  and 
the  disturbances  of  sensation  (the  loss  of  pain  and  temperature  sense,  while 
the  tactile  and  muscular  sense  are  preserved)  are  very  typical  of  syringo-my- 
elia and  are  rarely  met  with  in  cases  of  spinal  tumor.  In  syringo-myelia 
there  is  also  evidence  in  many  cases  of  a  slowly  ascending  or  descending 
morbid  process  and  such  slow  ascent  or  descent  is  not  common  in  cases  of 
tumor.     The  differential  diagnosis  is  one  of  great  importance,  for  the  one 


TUMORS   OF   THE   SPINAL    CORD  AND  ITS  MENINGES.       327 

disease  is  compatible  with  prolongation  of  life,  while  the  other  is  rapidly  de- 
structive.    (See  end  of  this  chapter.) 

The  symptoms  of  neuritis  may  for  a  time  obscure  those  due  to  tumor. 
In  cases  which  I  have  observed  the  first  symptoms  that  appeared  were  those 
of  a  neuritis  involving  various  branches  of  the  brachial  plexus.  These  were 
due  to  compression  of  the  spinal  roots,  and  are  naturally  present  in  those 
cases  in  which  the  tumor  begins  either  in  the  meninges  or  near  the  surface 
of  the  cord.  The  early  appearance  of  complete  paralysis  and  the  develop- 
ment of  other  symptoms,  particularly  those  pointing  to  interference  with  the 
vesical  and  rectal  reflexes,  will  argue  in  favor  of  neoplasm  and  not  of  a  sim- 
ple neuritis.  The  rapid  progress  of  the  symptoms  in  the  case  of  tumor,  the 
entire  absence  of  fever  in  most  of  them,  will  also  point  to  tumor  rather  than 
to  neuritis.  The  presence  of  sensory  and  paralytic  symptoms  in  both  halves 
of  the  body,  or  in  the  lower  as  well  as  upper  extremities,  without  involvement 
of  the  bladder  or  rectum,  may  be  considered  as  pointing  to  a  multiple  neuritis 
rather  than  to  tumor. 

A  localized  specific  meningitis  may  at  times  simulate  tumors  of  the  cord, 
and,  as  was  indicated  before,  the  frequent  association  of  gummatous  de- 
posits with  such  specific  meningo-myelitis  may  present  unusual  difficulties  of 
differential  diagnosis.  In  one  case  under  my  observation  for  a  period  of 
several  months,  it  was  impossible  to  differentiate  accurately  between  these 
two  conditions ;  and  unfortunately  the  result  of  treatment  is  not  to  be  de- 
pended upon,  for  even  in  cases  of  a  distinctly  specific  character  the  symptoms 
do  not  always  yield  to  antisyphilitic  treatment. 

Severe  injuries  of  the  spinal  cord  may  present  symptoms  resembling  those 
of  tumor.  But  the  history  of  the  case,  and  the  extreme  painfulness  over 
the  spinous  processes,  will  prevent  the  possibility  of  confusion  between  the 
two. 

Prognosis. —  In  spite  of  increasing  surgical  successes 
tumor  of  the  cord  is  an  extremely  grave  disease.  Spinal 
growths  are  secondary  to  malignant  disease  in  other  parts 
of  the  organism  and  help  to  intensify  the  gravity  of  the  dis- 
ease elsewhere.  In  the  case  of  tubercle,  sarcomata,  and  car- 
cinomata  the  prognosis  is  absolutely  bad,  while  it  is  only  a 
little  better  in  cases  of  gummata. 

The  duration  of  the  disease  varies  according  to  the  site 
of  the  tumor  and  according  to  the  morbid  character  of  the     v 
new  growth.     Tumors  of  the  cervical  region  are,  on  the     v 
whole,  more  rapidly  fatal  than  those  in  the  dorsal  or  lumbar 
region.    Tubercles,  carcinomata,  and  sarcomata,  take  a  mo^- 
rapid  course,  as  a  rule,  than  the  other  forms  of  neoplasm.'- 

Treatment.  —  Tumors  of  the  spfnal  cord  ancWts  me- 
ninges cannot  be  sensibly  affected  by  drugs.   ^T-here  is  a 


< 


328  THE  NERVOUS  DISEASES   OF   CHILDREN. 

possible  exception  in  the  case  of  guramata ;  yet,  as  was 
intimated  above,  the  effect  of  specific  treatment  in  these 
cases  is,  to  say  the  least,  extremely  doubtful.  I  would  ad- 
vise, however,  that  every  case  of  tumor  of  the  cord  be 
given  the  benefit  of  the  doubt,  and  that  active  specific 
treatment  by  iodides  and  mercurial  inunctions  be  attempted. 
If  these  drugs  avail  little,  no  other  drug  need  be  exhibited. 

Much  has  been  expected  of  surgical  treatment;  contrary 
to  our  experience  with  brain  tumors,  these  expectations 
are  nearer  fulfilment.  Extra-medullary  growths  are  the 
only  ones  that  we  can  expect  the  surgeon  to  remove.  Suc- 
cess has  been  reported  by  Gowers,  Horsley,  Starr,  Bruns, 
Oppenheim,  Spiller,  myself,  and  others.*  The  cases  most 
favorable  for  operation  are  those  which  occur  near  the  sur- 
face of  the  cord  or  in  the  meninges,  and  those  in  which  the 
operation  is  done  at  a  relatively  early  day,  before  the  neo- 
plasm has  invaded  the  cord  or  has  been  followed  by  sec- 
ondary myelitis  and  secondary  degenerations.  The  cases 
published  by  Laqueur,  in  which  the  tumor  was  found  out- 
side of  the  dura  mater  and  compressing  the  cauda  equina, 
those  reported  by  Oppenheim,  Dejerine,  Fraenkel,  Putnam, 
Boettiger-Krause,  and  myself,  make  it  almost  incumbent 
upon  us  to  urge  operation  in  extra-spinal  tumors. 

The  question  arises  whether  extirpation  of  the  tumor 
should  be  attempted  in  cases  of  a  tubercular  nature  and 
in  those  in  which  gumma  is  suspected.  In  tubercle  of  the 
cord,  even  the  successful  removal  would  in  all  probability 
prolong  life  but  very  little,  so  that  surgical  interference  is 
scarcely  warranted ;  and  yet  in  a  case  that  is  otherwise  fa- 
vorable for  operation  there  seems  to  me  to  be  sufficient  rea- 
son to  interfere,  if  there  are  no  other  symptoms  of  general 
tuberculosis.  I  am  in  favor  of  removing  gummata  if  the 
tumor  has  resisted,  as  it  generally  does,  every  form  of  treat- 
ment, provided  such  treatment  has  been  carried  out  relig- 
iously for  a  period  of  from  three  to  four  weeks  without  any 
sign  of  improvement.  I  consider  Horsley 's  advice  entirely 
sound,  that  if  a  tumor  has  resisted  every  form  of  treatment 
for  a  sufficient  length  of  time,  and  if   the  conditions  are 

*  Oppenheim  states  that  in  17  of  46  tabulated  cases  (36  per  cent.)  cure  or  improve- 
ment was  obtained  as  a  result  of  surgical  interference. 


TUMORS   OF  THE  SPINAL    CORD  AND  ITS  MENINGES.      329 

favorable  for  surgical  interference,  the  proper  surgical  pro- 
cedures should  be  resorted  to,  and  that,  too,  without  much 
delay,  for  delay  is  far  more  serious  under  these  circum- 
stances than  prompt  and  proper  surgical  interference  would 
be. 

SYRINGOMYELIA   AND   GLIOSIS  OF  THE  CORD. 

Syringomyelia,  or,  more  properly  speaking,  "  myelosyringosis,"  is  a  form 
of  disease  that  is  rare  enough  in  the  adult,  and  still  rarer  in  children.  Its 
symptoms  resemble  somewhat  those  of  tumor  of  the  cord,  of  amyotrophic  lat- 
eral sclerosis  and  of  progressive  muscular  atrophy.  As  the  name  indicates, 
the  disease  is  due  to  cavity  formation  in  the  spinal  cord.  This  may  be  the 
result  of  a  congenital  abnormality  of  structure,  or  it  may  be  due  to  a  glioma- 
tous  process  starting  in  the  vicinity  of  the  central  canal,  and  causing  a  de- 
struction of  tissue.      (See  monographs  by  Hoffmann  and  Schlesinger.) 

We  need  not  discuss  whether  the  one  or  the  other  mode  of  origin  is  the 
more  frequent,  and  on  this  point  opinions  still  vary.  The  cavity  is,  as  a  rule, 
largest  in  the  cervical  segments,  but  may  be  continued  up  into  the  medulla  and 
downward  into  the  dorsal  and  lumbar  segments. 

Symptoms. — Less  than  fifteen  years  ago  syringomyelia  was  considered  a 
mere  curiosity  without  any  special  practical  importance  ;  but  the  investigations 
of  Schultze  and  of  Kahler  have  enabled  us  to  recognize  the  disease  by  a  very 
definite  set  of  symptoms.  The  symptoms  will  naturally  vary  according  to  the 
extent  of  the  cavity  ;  but  as  the  cavity  is  generally  most  developed,  and  has 
its  beginning  in  the  cervical  region,  the  symptoms  are  first  observed  in  the 
upper  extremity  and  generally  around  the  shoulder. 

We  may  divide  the  symptoms  into  three  groups  :  The  first  group  consists 
of  trophic  disturbances,  affecting  the  skin,  its  subcutaneous  tissues,  and  the 
bones.  Glossiness  of  skin,  particularly  of  the  fingers,  deep  fissures,  felons — ■ 
which  are  sometimes  painless — and  phalangeal  necrosis,  with  marked  distor- 
tion of  the  fingers,  are  the  most  frequent.  Patients  also  complain  of  burning, 
prickling  feelings,  and  of  a  sensation  of  numbness.  These  various  disturb- 
ances are  associated  at  an  early  date  with  the  second  group  of  symptoms,  con- 
sisting of  partial  disturbances  of  sensation.  There  is  either  a  diminution  or  a 
complete  abolition  of  the  sense  of  pain  and  of  temperature,  while  the  sense  of 
touch  and  of  muscular  innervation  remains  absolutely  normal. 

The  third  group  of  symptoms  includes  a  progressive  atrophy  of  the  mus- 
cles, beginning  in  the  small  muscles  of  the  hand,  and  gradually  involving  the 
forearm,  the  arm,  and  the  shoulder  muscles.  The  resemblancce  to  the  Aran- 
Duchenne  type  of  progressive  muscular  atrophy  is  very  great  (see  Chapter 
XXII),  and  it  is  only  by  the  association  of  this  progressive  muscular  atrophy 
with  dissociated  disturbances  of  sensation,  and  with  the  trophic  symptoms 
described  above,  that  we  can  differentiate  between  syringomyelia  and  progres- 
sive muscular  atrophy,  as  well  as  amyotrophic  lateral  sclerosis.  In  progressive 
muscular  atrophy  of  the  Aran-Duchenne  type,  there  are  no  marked  disturb- 
ances in  the  skin  and  subcutaneous  tissue  ;  and  in  amyotrophic  lateral  scle* 


330  THE  NERVOUS  DISEASES   OF   CHILDREN. 

rosis  there  are  no  disturbances  of  sensation,  and  no  marked  trophic  symptoms, 
but  in  this  disease  and  in  syringomyelia  the  reflexes  may  be  exaggerated. 

In  syringomyelia  other  symptoms  occur,  which  will  depend  largely  upon 
the  extent  of  the  cavity  formation.  If  the  cavity  encroaches  very  largely 
upon  the  anterior  gray  matter,  there  will  be  a  large  amount  of  flaccid  atrophy 
and  paralysis,  the  electric  reactions  will  be  disturbed,  and  the  reflexes  will  be 


Fig.  76. — Section  through  Seventh  Cervical  Segment,  showing  Gliosis  of  Cord. 

(Herter.)* 

diminished  in  the  parts  governed  by  the  affected  region.  Fibrillary  tremor  is 
present  in  some  cases.  All  these  symptoms  are  generally  most  pronounced 
in  the  upper  extremities,  since  the  cavity  is  greatest  in  the  cervical  region,  and 
if  the  lateral  columns  in  the  cervical  region  are  involved,  we  may  expect  a  spastic 
paralysis  of  the  lower  extremities  with  increased  reflexes,  rigidity,  and  con- 
tractures. Cutaneous  reflexes  are,  at  times,  normal,  at  other  times  diminished 
or  entirely  absent.  Hyperidrosis  or  hypidrosis  (unilateral  or  bilateral)  have 
been  reported.     In  the  cervical  cases  a  narrowing  of  the  palpebral  fissure  and 

*  This  and  the  following  figure  are  reproductions  of  specimens  prepared  and  pho- 
tographed by  Dr.  Herter,  and  kindly  furnished  me  for  use  in  this  book. 


TUMORS   OF   THE   SPINAL    CORD   AND   ITS  MENINGES.       33  r 

contraction  or  dilatation  of  the  pupils  must  be  added  to  the  possible  symptoms 
of  this  curious  disease.  If  the  affection  extends  into  the  medulla  and  pons, 
disturbed  sensation  in  the  area  supplied  by  the  trigeminus  is  present,  together 
with  atrophy  and  paralysis  of  the  tongue,  hoarseness,  difficulty  of  deglutition, 
and  disturbances  of  the  senses  of.  taste  and  hearing.  Other  cranial  nerve 
nuclei  may  be  involved  ;  nystagmus  may  be  present ;  polyuria  and  increased 
salivation  have  also  been  noticed.     Pains  and  rigidity  in  the  upper  portion  of 


Fig.  77.— Section  through  Part  of  Fourth  Dorsal  Segment.  The  central  canal  is  in- 
vaded by  glia  cells  and  surrounded  by  groups  of  cells  and  nuclei  resembling  those  of 
the  ependyma.     (Herter.) 

the  spinal  column  are  not  infrequent,  and  scoliosis  or  a  combination  of  scoli- 
osis and  kyphosis  may  also  be  present.  Vesical  and  rectal  disturbances  are 
at  times  superadded. 

Syringomyelia  is  practically  a  disease  of  adolescence  and  of  adult  life,  but 
some  of  its  symptoms  can  at  times  be  traced  back  to  the  second  decade  of 
life.  The  earliest  case  which  I  have  seen  was  in  a  woman  of  twenty,  in 
whom  the  first  symptoms  began  at  the  age  of  fifteen.  It  is  interesting  to 
note,  however,  in  this  connection,  that  a  disease  which  is  intimately  related 


332  THE   NERVOUS  DISEASES    OE   CHILDREN. 

(at  least  in  its  pathology)  to  syringomyelia,  has  been  observed  by  Dr.  Herter 
in  a  child  one  year  of  age.  Through  his  kindness  I  am  enabled  to  insert  a 
summary  of  his  (unpublished)  case,  which  he  reported  to  the  New  York  Neu- 
rological Society. 

Three  months  before  the  child  was  examined  the  right  arm  and  hand 
began  to  be  weak,  and  the  head  drooped  forward.  The  examination  brought 
out  the  following  points  :  Complete  loss  of  power  and  atrophy  of  muscles  in 
both  arms  and  shoulders  ;  the  muscles  were  flabby  ;  there  was  slight  move- 
ment of  hands  and  fingers  ;  rigidity  of  neck  ;  the  knee-jerks  were  increased, 
especially  on  the  right  side  ;  ankle  clonus  was  elicited  on  both  sides  ;  there 
was  an  irregular  rise  of  temperature,  ranging  between  ioo°  and  1050  F.  daily. 
The  child  died  two  weeks  after  examination. 

The  post-mortem  examination  revealed  considerable  enlargement  of  the 
right  half  of  the  medulla  oblongata  and  of  the  cervical  cord  extending  to  the 
eighth  cervical  segment.  There  was  some  enlargement  from  this  point  to  the 
sixth  dorsal ;  below  this  the  cord  appeared  to  be  normal.  In  the  upper  cer- 
vical segments  the  nervous  structures  of  the  spinal  cord  were  replaced  by  a 
new  growth  ;  in  the  lower  cervical  region  the  pressure  effects  of  the  new 
growth  were  evident.  At  about  the  level  of  the  seventh  cervical  segment 
the  central  canal  was  lined  with  an  unbroken  layer  of  columnar  ependymal 
cells,  except  at  the  posterior  wall,  which  was  broken  through  by  a  mass  of 
larger  glia  cells,  which  partially  filled  the  canal.  (Fig.  yy.)  The  glioma  in- 
vaded nearly  the  entire  cross-section  of  the  cord,  but  undoubtedly  originated 
in  the  vicinity  of  the  central  canal. 

The  case  is  not  unlike  one  reported  by  K.  Miura ;  Sokoloff  described  a 
glioma  of  the  medulla,  with  cavity  formation,  in  a  boy,  aged  five  years. 


CHAPTER   XX. 

FAMILY  DISEASES   OF   THE   SPINAL   OR   CEREBRO-SPINAL 

SYSTEM. 

Family  affections  of  the  entire  central  nervous  system 
constitute  an  important  group  of  diseases.  They  are  the  re- 
sult of  defective  development,  or  possibly  of  early  degener- 
ation of  various  parts  and  tracts  in  the  brain  and  spinal  cord. 
Of  these  disorders  hereditary  ataxy,  or  Friedreich's  disease, 
is  the  best  known.  A  few  years  ago  Nonne  and  Marie  de- 
scribed a  type  of  disease  which  bears  a  close  resemblance 
to  hereditary  ataxy,  and  for  which  Marie  proposed  the 
term  Hereditary  cerebellar  ataxy  (Heredo-ataxie  cerebel- 
leuse).  In  Friedreich's  disease  the  morbid  process  is  re- 
stricted largely  to  the  posterior  or  to  the  postero-lateral  col- 
umns ;  in  Marie's  disease  there  is  in  all  probability  a  defec- 
tive development  of  the  cerebellum  and  of  the  tracts  con- 
nected with  it.  Congenital  or  hereditary  defect  of  the 
pyramidal  tracts  also  leads  to  another  distinct  group  of  dis- 
eases which  we  may  term  Hereditary  spastic  paralysis  ;  and 
Hoffman  has  still  more  recently  described  an  hereditary 
form  of  progressive  muscular  atrophy  of  spinal  origin  in 
which  the  chief  defect  was  found  in  the  anterior  gray  mat- 
ter of  the  cord.  These  hereditary  diseases  suggest  a  clinical 
relationship  to  syphilitic  disease  of  the  spinal  cord  (which 
may  be  inherited,  though  not  hereditary)  and  to  sOme  forms 
of  cerebral  birth  palsies.  Moreover,  some  of  these  hered- 
itary affections  are  distinctly  cerebro-spinal,  both  as  regards 
the  distribution  of  the  lesions  and  the  character  of  the  symp- 
toms. It  is  no  easy  task  to  separate  these  various  forms 
from  one  another  and  to  bring  order  out  of  chaos.  The 
family  form  of  progressive  muscular  atrophy  will  be  con- 
sidered to  much  greater  advantage  in  connection  with  the 
other  forms  of  progressive  muscular  disease. 


334 


THE   NERVOUS   DISEASES    OF   CHILDREN, 


HEREDITARY   ATAXY. — FRIEDREICH'S   DISEASE. 

Hereditary  ataxy,  or  Friedreich's  disease,  as  it  is  called 
in  honor  of  the  physician  who  gave  the  first  satisfactory  ac- 
count of  it,  is  a  family  af- 
fection. Several  genera- 
tions are  often  affected  by 
it ;  but  naturally  we  may 
find  every  now  and  then 
that  the  disease  occurs 
in  one  or  several  mem- 
bers of  a  family  without 
any  history  of  a  similar 
trouble  in  the  patient's 
ancestry.  It  is  not  nec- 
essary, therefore,  to  speak 
of  such  cases  as  non- 
hereditary  forms  of  Fried- 
reich's ataxia,  as  an  Eng- 
lish writer  has  recently 
done,  for  the  disease  must 
invariably  have  a  starting- 
point,  and  the  patients 
under  observation  may 
indeed  be  the  first  of  a 
long-  series  that  are  to 
follow.  The  children  who 
have  come  under  my  spe- 
cial notice  in  three  dif- 
ferent families  gave  no 
history  of  any  similar 
trouble  in  preceding  gen- 
erations. 

The  marked  tenden- 
cy to  the  occurrence  of 
this  disease  in  families  is 
shown,  as  Gowers  states, 
by  the  occurrence  of  sixty-five  cases  in  nineteen  families, 
c-iving  an  average  of  rather  more  than  three  to  each  fam- 


Fig.  78.— Case  of  Hereditary  Ataxy   (Fried- 
reich's Disease). 


HEREDITARY  DISEASES   OF   THE   SPINAL    CORD. 


335 


ily.  The  number  has  varied  from  two  to  as  many  as  eight 
in  a  single  family.  Both  sexes  are  about  equally  liable,  but 
sometimes  a  special  predilection  is  'shown  in  favor  of  one 
or  the  other  sex.  In  one  family  of  nineteen,  two  males  were 
affected  with  the  disease  and  seventeen  females  escaped. 
All  the  cases  which  I  have  observed  were  in  boys,  the  sis- 
ters of  these  escaping  entire- 
ly unharmed.  The  disease 
comes  on,  as  a  rule,  very  early 
in  life,  most  of  the  symptoms 
being  fully  developed  before 
the  age  of  fourteen.  In  the 
family  referred  to  by  Everett 
Smith,  of  Boston,  the  symp- 
toms of  ataxic  paraplegia 
were  observed  in  the  father 
at  the  age  of  sixty-six,  but  it  is 
questionable  whether  his  was 
a  typical  hereditary  ataxy, 
and  it  is  safer  to  adhere  to 
the  belief  that  Friedreich's 
disease  will  always  appear 
early  in  life.  If  several  mem- 
bers of  one  family  are  affected 
the  disease  is  developed  in 
all  at  about  the  same  age. 

Symptoms.  —  The  symp- 
tomatology of  hereditary  at- 
axy is  now  as  firmly  estab- 
lished as  that  of  almost  any 
spinal  disease.  Friedreich 
described  most  of  the  symp- 
toms in  1861.  The  French 
school,  including  Charcot, 
Vulpian,  Brissaud,  and  Ma- 
rie, have  given  careful  study  to  this  disease.  In  England, 
Carpenter,  Gowers,  Ormerod,  and  Bury  described  cases  of 
true  hereditary  ataxy  ;  while  in  this  country  the  chief  con- 
tributions to  this  subject  have  been  made  by  Smith,  Ham- 
mond, Seguin,  Dana,  Church,  Sanger  Brown,  and  Inglis. 


Fig.  79. — Same  Patient  as  in  Fig.  78. 
Marked  atrophy  of  the  muscles  about 
the  shoulder  girdles  (infra-spinati,  su- 
pra-spinati,  rhomboids,  and  others). 


336  THE  NERVOUS  DISEASES   OF   CHILDREN. 

At  a  very  early  age  children  suffering  from  Friedreich's 
disease  exhibit  peculiarities  in  walking  and  standing.  The 
child  walks  with  its  legs  widely  apart,  in  an  uncertain,  hes- 
itating fashion,  reminding  one  very  strongly  of  a  combined 
ataxic  and  cerebellar  gait.  There  are  in  addition  in  some  pa- 
tients oscillatory  movements  of  the  head,  which  remind  one 
a  little  of  multiple  sclerosis,  and  a  little  of  such  movements 
as  we  sometimes  see  in  cases  of  senile  degeneration.  When 
the  patient  is  asked  to  stand  still,  with  his  feet  closely  ap- 
proximated, he  soon  begins  to  stagger,  as  tabic  patients  do, 
and  as  soon  as  his  eyes  are  closed  falls  to  the  ground  un- 
less properly  supported.  Marie  states  that  Romberg's 
symptom  is  ordinarily  absent,  but  it  has  been  most  distinct- 
ly present  in  those  subjects  whom  I  have  had  occasion  to 
examine.  In  addition  to  these  disturbances  in  gait  and  in 
standing  we  also  find  a  coarse  tremor  present  in  many  of  the 
cases,  and  in  some  a  condition  which  is  not  so  distinctly 
tremulous  as  it  is  ataxic  and  awkward.  If  the  patient  is 
asked  to  take  hold  of  a  pencil,  to  raise  a  glass  of  water  to 
his  lips,  or  to  attempt  to  write,  he  seizes  upon  the  object 
that  he  is  to  hold  in  a  distinctly  ataxic  way  and  uses  the 
hands  awkwardly,  sometimes  after  the  fashion  of  extremely 
choreic  movements.  It  is  this  combination  of  tremor,  of 
awkward  movements,  and  of  choreic  manifestations  that 
gives  to  these  children  their  characteristic  aspect.  There 
has  been  some  discussion  as  to  the  occurrence  of  actual  pa- 
ralysis ;  in  those  whom  I  have  examined  there  was  in  the 
first  few  years  only  a  very  slight  loss  of  muscular  power 
both  in  the  upper  and  in  the  lower  extremities.  In  ad- 
vanced stages  the  grasp  is  almost  nil,  and  the  lower  extrem- 
ities may  be  so  thoroughly  paralyzed  that  it  is  impossible 
for  the  patient  to  lift  a  leg  or  even  to  move  a  toe,  but  in  this 
respect  the  disease  evidently  varies  very  much. 

Sensation  may  be  interfered  with  to  a  slight  degree,  as  is 
maintained  by  Senator,  Oppenheim,  Rutimeyer,  Soca,  and 
others.  Lightning  pains  are  infrequent,  although  Charcot  in- 
sists that  in  some  instances  these  occur  quite  as  typically  as 
in  tabes.  Anaesthesia  and  analgesia  are  rarely  present.  Sev- 
eral French  authors  have  described  cases  in  which  hemi- 
ansesthesia  occurred;  but  Marie  is  inclined  to  attribute  this 


HEREDITARY  DISEASES   OE   THE  SPINAL    CORD.        337 

to  the  association  of  hysteria  with  Friedreich's  disease.  The 
muscular  sense  is  rarely  affected,  and  in  the  persons  whom 
I  have  examined  for  the  purpose  of  determining  the  pres- 
ence or  absence  of  this  sense,  I  have  found  it  invariably 
present,  even  in  those  in  whom  there  was  marked  inco- 
ordination of  the  upper  extremities. 

Reflexes. — The  superficial  reflexes  are,  as  a  rule,  pre- 
served ;  but  the  deep  reflexes  are  generally  absent.  The 
Babinski  reflex  is  often  present.  The  absence  of  the  knee- 
jerks  brings  out  in  some  subjects  the  very  closest  resem- 
blance between  Friedreich's  disease  and  tabes  dorsalis.  In 
cases  in  which  the  reflexes  are  exaggerated  there  is  good 
reason  to  doubt  whether  the  disease  corresponds  to  that 
described  by  Friedreich;*  but  there  is  no  reason  why  in 
those  forms  in  which  the  affection  involves  both  the  poste- 
rior and  lateral  columns  the  knee-jerk  should  not  occasion- 
ally be  augmented,  and  it  is  probably  in  view  of  such  mixed 
types  that  Gowers  has  spoken  of  a  form  of  hereditary  ataxic 
paraplegia  under  the  heading  of  Friedreich's  disease.  It  is 
one  thing  to  maintain  a  type  as  originally  described  by  its 
discoverer,  and  quite  another  thing  to  deny  that  any  de- 
parture from  such  a  typical  series  of  symptoms  implies  an 
entirely  different  order  of  disease. 

Difficulties  of  micturition  and  of  defecation  occasionally 
occur,  but  are  not  so  constant  an  accompaniment  of  Fried- 
reich's disease  as  they  are  of  tabes.  Profuse  salivation 
and  disturbances  in  respiration  (Oppenheim)  have  been 
recorded. 

Trophic  and  vasomotor  disturbances  are  also  rare,  if  we 
except  a  general  discoloration  of  the  skin  and  coldness  of 
feet  and  hands.  Marie  and  other  French  authors  refer  to 
the  occurrence  of  a  special  form  of  club-foot  which  they 
have  observed  in  these  cases  of  hereditary  ataxy.  Marie 
states  that  the  foot  is  shortened  ;  that  the  anterior  portion 
is  particularly  broad  if  viewed  laterally ;  the  foot  is  in  the 
condition  of  a  pes  cavus.  The  toes  are  hyperextended  and 
have  a  claw  shape.  This  deformity  of  the  toes  is  said  to 
have  been  observed  as  one  of  the  early  symptoms  by  parents 
in  whose  families  this  special  disease  has  been  hereditary. 

*  Some  of  these  may  belong  to  the  Heredo-ataxie  cerebelleuse  of  Marie. 


338 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


Muscular  atrophy  constitutes  an  important  symptom  of 
this  disease.  It  is  most  distinctly  visible  in  the  shoulder 
and  pelvic  girdles.  It  was  so  prominent  a  feature  in  one  of 
my  patients  that  on  first  examination  I  was  inclined  to  re- 
gard the  trouble  as  one  of  an  hereditary  form  of  progressive 
muscular  atrophy,  until  the  examination  of  the  brother,  who 
was  in  a  more  advanced  stage  of  the  disease,  proved  to  me 
beyond  the  possibility  of  a  doubt  that  both  cases  were  typ- 
ical of  Friedreich's  disease.  In  the  boy  represented  in  Fig. 
90,  the  excessive  atrophy  must  be  attributed  to  an  addi- 
tional involvement  of  the  gray  matter  of  the  cord. 


Fig.  80. — Deformity  of  the  Feet  in  a  Case  of  Friedreich's  Disease  ;  Hyperextension 
of  the  Toes  and  Club  Foot.     (Marie.) 


We  have  now  to  consider  a  further  set  of  symptoms 
which  seem  to  me  to  prove  the  cerebro-spinal  character  of 
the  disease.  Nystagmus  occurs  in  many  of  these  cases,  but, 
as  a  rule,  does  not  appear  until  several  years  after  the  onset 
of  the  first  symptoms.  The  nystagmus  can  often  be  elicited 
only  upon  extreme  use  of  the  ocular  muscles.  In  this  con- 
nection it  may  be  well  to  remember  that  nystagmus  upon 
extreme  movements  occurs  in  not  a  few  healthy  individuals, 
and  that  the  presence  of  such  movements  in  a  child  of  a 
family  affected  with  Friedreich's  disease  need  not  neces- 
sarily indicate  the  development  of  this  serious  trouble. 
Ocular  palsies  occur,  but  are  rare.      The  Argyll-Robertson 


HEREDITARY  DISEASES   OF   THE   SPINAL    CORD.        339 

pupil  is  as  regularly  absent  in  Friedreich's  disease  as  it  is 
present  in  cases  of  tabes.  Optic  atrophy  and  amblyopia 
have  not  been  described  in  these  cases.  All  the  other 
senses  remain  entirely  unaffected. 

Among  cerebral  disorders  occurring  in  the  course  of 
Friedreich's  disease,  vertigo  and  dizziness  are  extremely 
frequent.  These  may  in  part  be  due  to  the  oscillatory 
movements  of  the  head.  In  the  earlier  years  of  the  disease 
the  intelligence  is  not  affected,  except  that  the  children  can- 
not be  educated  at  schools  as  other  children  are,  and  there- 
fore remain  backward.  As  the  disease  progresses  a  distinct 
defect  of  intelligence  is  noticeable,  and  a  condition  of  semi- 
idiocy  may  be  the  result.  The  patients  appear,  however, 
far  more  idiotic  than  they  really  are,  and  this  appearance  is 
caused  by  the  peculiar  stupid  expression  of  face,  and  above 
all  by  the  disturbances  in  speech,  to  which  we  shall  now 
refer.  The  speech  reminds  one  a  little  of  multiple  sclerosis 
in  that  it  is  slightly  scanning,  but  it  is  at  times  hesitating 
and  jerky,  at  other  times  slow,  deliberate,  and  awkward. 
On  the  whole,  I  find  Marie's  simile  a  good  one,  who  com- 
pares it  with  the  cerebellar  gait,  since  it  is,  as  he  says,  awk- 
ward, uncertain,  and  vacillating.  All  these  symptoms  are 
not  developed  until  after  the  lapse  of  years.  At  first  the 
peculiar  gait  and  position  are  noticeable,  later  on  the  inco-# 
ordination  of  the  upper  extremities  and  the  difficulties  of 
speech  become  more  marked  ;  then,  after  the  lapse  of  a 
longer  or  shorter  period  of  time,  the  patients  become  help- 
less and  may  remain  bedridden  or  confined  to  their  chairs 
for  a  period  of  ten  and  even  twenty  years. 

Differential  Diagnosis. — Attention  must  be  directed 
to  the  following  points  : 

Tabes.  Friedreich's  Disease. 

Argyll-Robertson  pupil  present.  Argyll-Robertson  pupil  absent. 

No  nystagmus.  Nystagmus  present  in  later  years. 

No  tremor  of  the  head.  Tremor  of  the  head  present. 

No  peculiarities  of  speech.  Speech  altered. 

Inco-ordination  of  upper  extremities  Inco-ordination  the  rule. 

rare. 

Lightning  pains  and  various  crises.  No  such  pains  and  crises,  as  a  rule. 

Ataxic  gait.  Ataxic-cerebellar  gait. 

No  defect  of  intelligence.  Intelligence  defective  in  later  years. 


340  THE  NERVOUS  DISEASES   OE   CHILDREN. 

In  the  above  parallel  columns  the  symptoms  which  both 
have  in  common  have  been  omitted,  such  as  absence  of  re- 
flexes and  Romberg's  symptom. 

Multiple  sclerosis  can  be  distinguished  easily  from 
Friedreich's  disease  by  the  excessive  exaggeration  of  the 
reflexes,  by  the  intention  tremor,  by  the  marked  spasticity 
of  the  gait,  by  the  occurrence  of  ocular  palsies,  all  of  which 
rarely  occur  in  cases  of  Friedreich's  disease.  Confusion 
might  arise  from  consideration  of  the  similarity  in  speech 
and  the  occurrence  of  nystagmus.  Marie  includes  in  the 
consideration  of  the  differential  diagnosis  ordinary  chorea, 
but  it  seems  to  me  that  no  one  but  a  very  obtuse  person 
could  be  misled  by  the  occasional  occurrence  of  awkward 
and  choreic  movements  in  Friedreich's  disease,  for  in  no 
other  respect  are  the  two  diseases  at  all  similar. 

Confusion  may  more  readily  occur  with  cerebellar  diseases,  and  Schultze 
some  time  ago,  in  answer  to  Senator's  observations,  called  attention  to  the 
fact  that,  after  all,  cases  that  would  seem  to  be  of  the  cerebellar  order  are 
altogether  different  from  the  type  which  Friedreich  described.  I  can  ap- 
prove of  this  remark  of  Schultze,  for  I  have  myself  seen  a  case  of  defective 
cerebellar  development*  in  which  there  was  a  superficial  resemblance  be- 
tween it  and  cases  of  Friedreich's  disease,  and  yet  a  careful  considera- 
tion of  the  conditions  was  sufficient  to  distinguish  between  the  two  sets  of 
diseases. 

Pathological  Anatomy.  —  The  morbid  anatomy  of 
Friedreich's  disease  has  not.  as  yet,  been  satisfactorily  de- 
termined. Many  autopsies  have  been  made,  but  in  some  of 
the  cases  which  have  been  examined  post  mortem,  the  symp- 
toms have  varied  so  much  from  the  type,  as  laid  down  by 
Friedreich,  that  the  results  of  the  examination  have  helped 
to  obscure  rather  than  to  clear  up  the  doubts  regarding  the 
true  anatomical  changes  of  the  disease.  A  number  of  au- 
thors have  called  attention  to  the  attenuation  of  the  entire 
cord  in  cases  of  hereditary  ataxia,  the  diameter  of  the  cord 
being  but  three-fourths  or  two-thirds  of  the  normal.  This 
attenuation  is  most  marked  in  the  dorsal  region ;  but 
whether  it  is  due  to  a  disappearance  of  nerve-fibres,  to  the 
retraction  of  sclerotic  tissue,  or  whether  it  is  simply  a  de- 
fect of  development,  has  not  been  clearly  made  out.     Any 

*  See  Chapter  on  Conditions  due  to  Defective  Development  of  the  Brain. 


HEREDITARY  DISEASES   OF   THE   SPINAL    CORD. 


34£ 


one  of  these  three  causes  may  be  operative  in  some  cases, 
or  a  co-operation  of  all  three  is  a  possibility  in  others.  The 
one  fact  that  is  absolutely  indisputable  is  that  on  micro- 
scopical examination  a  sclerosis  of  the  spinal  cord  is  found, 
involving  at  different  levels,  or  at  one  and  the  same  level, 
various  systems  of  the  cord.  This  sclerosis  affects  most 
frequently  the  posterior  columns  or  the  lateral  columns,  or 
both  together,  and  hence  the  symptoms  vary  between  those 
of   a  pure  posterior  spinal    sclerosis   and   those  due  to  a 


Fig.  81.— Section  through  Middle  Dorsal  Region  of  a  Case  of  Friedreich's  Disease. 
(After  Blocq  et  Marinesco,  from  Marie's  treatise.)  A,  small  degenerated  area,  prob- 
ably Gowers'  tract ;  B,  degeneration  in  lateral  columns  ;  C,  direct  cerebellar  tract ; 
D,  column  of  Burdach ;  E,  column  of  Goll  —  both  degenerated  ;  G,  a  strand  of 
healthy  fibres. 


postero-lateral  sclerosis,  resembling  the  symptoms  of  the 
ataxic  paraplegia  of  the  adult. 

Taking  up  the  lesions  of  Friedreich's  disease  in  detail, 
we  may  note  the  following  distribution : 

I.  In  the  posterior  columns,  in  a  number  of  the  cases,  the  columns  of 
Goll  are  sclerosed  throughout  their  entire  length  from  the  lower  part  of  the 
spinal  cord  to  the  calamus  scriptorius.  The  column  of  Burdach  is  also  in- 
volved throughout  the  greater  part  of  its  course  ;  but,  according  to  Marie,  the 
intensity  of  this  sclerosis  varies  in  different  levels  of  the  cord.  The  external 
portion  of  the  column  of  Burdach,  near  the  posterior  horns,  is  generally  ex- 
empt frdm  sclerosis ;  in  the  cervical  region  the  sclerosis  of  the  column  of 
Burdach  begins  to  diminish,  and  disappears  altogether  in  the  vicinity  of  the 
medulla  oblongata. 


342 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


2.  The  direct  cerebellar  tract  is  involved  from  its  beginning  in  the  lower 
dorsal  region  to  the  upper  cervical  region.  Toward  this  latter  part  it  dimin- 
ishes considerably,  but  is  most  marked  in  the  upper  dorsal  segments.  Sev- 
eral observers  have  noted  that  the  lesion  extends  beyond  the  direct  cerebellar 
tract,  and  involves  the  antero-lateral  tract,  or  tract  of  Gowers ;  and  Marie 
goes  to  the  extent  of  stating  that  he  thinks  this  involvement  of  the  antero- 
lateral tract  an  almost  constant  feature  of  Friedreich's  disease. 

3.  As  for  the  lateral  columns,  the  opinions  of  authors  differ  somewhat. 
Marie  is  not  willing  to  allow  the  regular  involvement  of  the  lateral  columns, 
while  he  concedes  that  the  diseased  fibres  occupy  the  position  of  the  crossed 
pyramidal  tract.     He  does  not  believe  that  they  represent  the  fibres  of  that 


Fig.  82  — Section  through  a  Cervical  Segment  from  a  Case  of  Friedreich's  Disease. 
(Schultze.)  Degenerated  areas  in  posterior  and  lateral  columns  and  in  anterior  col- 
umn (left  half  of  figure).     Posterior  root  fibres  also  degenerated. 


tract.  His  reasons  for  maintaining  this  are,  first,  that  the  lesion  of  the  lateral 
columns  in  Friedreich's  disease  diminishes  considerably  from  below  upward  to 
the  level  of  the  lower  portion  of  the  medulla  oblongata,  the  very  reverse  of 
which  would  occur  if  these  fibres  were  part  of  the  pyramidal  tract.  Secondly, 
that  on  a  transverse  section  of  the  spinal  cord  the  localization  of  the  lesion 
of  the  lateral  column  does  not  correspond  exactly  to  the  site  of  the  lesion  of 
the  pyramidal  tract  proper.  Third,  nothing  in  the  clinical  appearances  of  Fried- 
reich's disease  reminds  one  of  the  symptoms  which  are  a  constant  accom- 
paniment of  changes  in  the  pyramidal  tract.  The  only  explanation  which 
Marie  is  able  to  give  for  the  fibres  that  are  affected  in  the  lateral  region  is 
that  they  are  probably  fibres  which  connect  the  direct  cerebellar  tract  with  the 
antero-lateral  tract  of  Gowers.  Of  the  reasons  advanced  by  Marie,  the  first 
seems  to  me  to  be  the  only  one  that  will  bear  close  scrutiny ;  the  second 
is  scarcely  susceptible  of  proof,  and  as  for  the  third,  it  is  sufficient  to  state 


HEREDITARY  DISEASES   OF   THE    SPINAL    CORD.         343 

that  the  disease  attacks  both  the  posterior  columns  and  the  pyramidal  fibres 
at  various  levels. 

4.  A  difference  of  opinion  also  exists  as  regards  the  marginal  zone  of 
Lissauer,  as  many  authors  claim  to  have  found  these  zones  entirely  intact, 
while  others  claim  that  an  affection  of  these  fibres  constitutes  a  regular  feat- 
ure of  Friedreich's  disease.  At  all  events  the  affection  of  Lissauer's  tract  is 
not  nearly  so  constant  or  so  early  a  feature  of  the  disease  as  it  is  in  the  spinal 
ataxia  in  the  adult. 

The  morbid  anatomy  of  Friedreich's  disease  is  not  exhausted  with  this 
statement  regarding  the  behavior  of  the  white  fibres.  The  gray  matter  is 
affected  as  well.  The  columns  of  Clarke  exhibit  a  loss  of  fibres  very  much 
like  that  in  tabes,  as  well  as  a  considerable  diminution  of  their  cells,  which 
are  also  smaller  and  devoid  of  their  long-cell  processes.  In  the  posterior 
horns  there  is  a  diminution  of  volume,  and  also  a  decided  diminution  in  the 
number  of  cells.  The  anterior  horns  have  been  found  altered  by  some, 
Friedreich  being  among  them,  and  the  cells  may  be  atrophied.  There  is 
no  reason  to  doubt  this  occurrence  in  view  of  a  considerable  atrophy  of  the 
muscles  which  occurs  in  some  of  these  cases,  as  in  one  of  my  own. 

Some  changes  have  been  noticed  in  the  ependyma  of  the  central  canal  and 
in  the  cerebellum  (Barker) ;  yet  these  are  not  regular  morbid  features  of 
Friedreich's  disease.  Some  have  also  insisted  that  the  spinal  meninges  are 
involved,  while  others  dispute  such  involvement.  If  they  are  affected  at  all 
it  is  in  the  vicinity  of  the  posterior  columns.  Little  more  can  be  said  of  the 
behavior  of  the  posterior  roots,  although  Blocq  and  Marinesco  insist  that  they 
are  as  frequently  altered  in  Friedreich's  disease  as  they  are  in  ordinary  tabes. 
The  peripheral  nerves  are  supposed  to  be  entirely  normal  in  Friedreich's  dis- 
ease, and  to  this  fact  Dejerine  and  others  attributed  the  entire  absence  of 
fulgurating  pains  in  hereditary  ataxy,  while  they  are  universally  present  in 
tabes  dorsalis. 

Senator  laid  especial  stress  on  the  congenital  atrophy  of  the  cerebellum 
and  considered  it  to  be  of  greater  importance  than  all  the  spinal  cord 
changes. 

The  question  remains  as  to  the  nature  of  the  morbid  proc- 
ess underlying  Friedreich's  disease?  Dejerine  and  Letulle, 
on  the  strength  of  certain  peculiar  fibres  which  they  have 
found,  and  which  they  consider  similar  to  fibres  discovered 
by  them  in  the  cortex  of  epileptic  patients,  are  inclined  to 
regard  the  sclerosis  of  hereditary  ataxia  as  a  sclerosis  of  the 
neuroglia.  They  insist  that  neither  the  connective-tissue 
septa  nor  the  blood-vessels  are  at  all  altered,  and  that  the  scle- 
rosis is  therefore  very  different  from  the  vascular  sclerosis 
which  so  often  involves  the  pyramidal  tracts  and  the  direct 
cerebellar  tract ;  but  later  investigators,  among  them  Wei- 
gert,  have  disproved  these  views  of  Dejerine  and  Letulle. 


344  THE   NERVOUS  DISEASES   OF   CHILDREN. 

They  regard  the  sclerosis  of  Friedreich's  disease  as  in  no- 
wise different  from  the  ordinary  sclerosis  excepting  in  this, 
that  it  has  been  developed  at  an  unusually  early  period. 
Weigert,  in  his  recent  studies  on  the  neuroglia,  concludes 
that  in  the  ordinary  spinal  sclerosis,  as  it  occurs  in  tabes,  in 
multiple  cerebro-spinal  sclerosis,  and  in  amyotrophic  lateral 
sclerosis,  the  proliferation  of  the  neuroglia  is  a  much  more 
marked  feature  than  in  hereditary  ataxy.  The  refutation 
of  this  theory  of  Dejerine  and  Letulle  does  not,  how- 
ever, help  us  in  the  recognition  of  any  true  theory  of  the 
disease,  and  for  the  present  we  must  confess  that  we  do  not 
know  why  or  how  the  sclerosis  of  Friedreich's  disease  is 
developed. 

Prognosis. — The  prognosis  of  Friedreich's  disease  is  in- 
variably grave.  In  the  course  of  four  to  six  years  the  patient 
becomes  crippled,  unable  to  move  about  except  in  a  chair, 
awkward  in  speech,  and  more  or  less  demented.  Unfortu- 
nately the  disease  does  not  lead  to  a  fatal  issue  within  a 
reasonable  period  of  time,  some  of  the  patients  attaining  the 
age  of  forty  years  and  more. 

As  for  treatment,  no  special  method  need  be  recom- 
mended. The  progress  of  the  disease  cannot  be  stayed,  the 
relief  of  pain  constituting  the  only  reason  for  active  inter- 
ference on  the  part  of  the  physician.  The  ordinary  anti- 
neuralgic  remedies  may  be  employed  to  this  end.  In  addi- 
tion, suspension,  electrical  treatment,  massage,  and  the  like, 
may  be  ordered,  in  the  discretion  of  the  physician. 

HEREDITARY   ATAXY   (CEREBELLAR  TYPE  ;   TYPE  NONNE-MARIE). 

Under  the  title  "  Heredo-ataxie  Cerebelleuse  "  Marie  collected  a  number 
of  cases  which  bear  a  very  close  resemblance  to  Friedreich's  disease,  yet  pre- 
sent several  characteristic  and  different  symptoms.  Titubation,  Romberg's 
symptom,  tremor  of  the  head  and  of  the  extremities,  choreiform,  awkward 
movements,  particularly  of  the  arms  and  hands,  and  nystagmus,  are  symp- 
toms common  to  Friedreich's  disease,  and  to  the  form  we  are  now  describing. 
The  cerebellar  form  of  hereditary  ataxy  comes  on,  as  a  rule,  at  the  age  of  pu- 
berty or  later.  The  knee-jerks,  instead  of  being  diminished,  are  increased. 
There  are  marked  ocular  symptoms,  the  pupils  fail  to  react  to  light  and  dur- 
ing accommodation.  There  is  diplopia  in  some  cases,  color-blindness  in. 
others.  Contraction  of  the  visual  field  has  been  observed,  and  amblyopia,  due 
to  atrophy  of  the  optic  nerve,  is  a  typical  symptom.     There  are  also  marked 


HEREDITARY  DISEASES   OF  THE  SPINAL    CORD.        345 

disturbances  of  sensation.  The  deformities  of  the  foot  and  kyphosis,  noted  in 
Friedreich's  disease,  are  never  present  in  the  cerebellar  type. 

This  symptomatology,  as  developed  by  Marie,  reveals  a  very  striking  re- 
semblance between  these  cases  of  hereditary  cerebellar  ataxy  and  a  form  of 
family  disease  described  by  Nonne.  In  all  probability  Nonne's  and  Marie'^ 
descriptions  refer  to  the  same  type.  While  the  latter  deserves  credit  for 
naming  the  disease,  it  seems  to  me  just  that  Nonne's  name  should  be  con- 
nected with  this  type.  There  is  also  a  very  close  resemblance  between  this 
hereditary  cerebellar  ataxy  and  some  of  the  cases  which  have  been  described 
under  the  heading  of  "  Hereditary  Spastic  Paralysis."  Senator's  views  regard- 
ing Friedreich's  disease  may  in  part  be  explained  by  the  recognition  of  this  spe- 
cial order  of  disease,  and  there  would  surely  be  a  possibility  of  confounding 
this  type  of  hereditary  ataxy  with  the  cerebellar  type  of  multiple  sclerosis 
which  Charcot  has  described. 

It  is  too  early  to  report  definitely  upon  the  pathology  of  hereditary  cere- 
bellar ataxy.  If  we  are  correct  in  considering  the  cases  described  by  Marie 
and  Nonne  as  representatives  of  the  same  type,  the  post-mortem  findings  in 
one  of  Nonne's  cases  will  give  some  clew  to  the  pathology  of  this  variety  of 
disease.  In  Nonne's  case  the  cerebellum  was  small,  and  there  was  a  simple 
diminution  of  tissue  without  sclerosis  or  degeneration.  The  spinal  cord  was 
much  attenuated  throughout  its  entire  length.  Under  the  heading  of  "  He- 
reditary Ataxy  and  Atrophy  of  the  Cerebellum  "  Menzel  described  a  condition 
in  which  there  was  a  combined  systemic  disease  of  the  spinal  cord,  and  in 
addition  atrophy  of  the  cerebellum,  pons,  and  medulla.  Two  French  authors, 
Royet  and  Collet,  have  reported  a  case  which  was  diagnosticated  during  life 
as  multiple  sclerosis,  in  which  there  was  found  on  post-mortem  examination 
a  lesion  of  the  cerebellum  and  of  the  cerebellar  tracts  in  the  pons.  It  is 
possible  that  some  of  the  cases  which  have  been  regarded  as  a  form  of  he- 
reditary multiple  sclerosis  may  come  under  this  same  heading.  I  recognize 
that  there  is  great  danger  in  multiplying  types  of  disease.  The  form  de- 
scribed by  Marie  and  Nonne  should  in  justice  to  these  two  authors  be  given 
due  consideration ;  but  I  believe  it  better  for  the  present  to  regard  it  as  a 
variety  of  hereditary  ataxy.* 


HEREDITARY     SPASTIC     PARALYSIS     (SPINAL,    CEREBRAL     OR 
CEREBRO-SPINAL  TYPES). 

During-  the  past  decade  or  two  several  family  affections 
have  been  described  by  Homen,  Newmark,  Pelizaeus,  Strum- 
pell,  Freud,  Erb,  and  myself,  in  all  of  which  a  spastic 
rigidity,  or  a  spastic  paralysis  affecting  the  lower  extremi- 
ties chiefly,  has  been  the  most  prominent  symptom.  In 
some  cases  the  spastic  symptoms  were  evidently  of  purely 

*  The  cases  reported  by  Dr.  Sanger  Brown  also  resemble  this  type. 


346  THE  NERVOUS  DISEASES   OF  CHILDREN. 

spinal  origin  ;  in  other  cases  they  were  associated  with  dis- 
tinct cerebral  symptoms.  A  classification  of  all  of  these 
various  diseases  according  to  the  morbid  conditions  under- 
lying them,  is  not  yet  possible ;  it  is  sufficient  for  the  pres- 
ent to  discuss  them  from  the  etiological  point  of  view.  I 
have  chosen,  therefore,  to  designate  them  as  hereditary 
spastic  paralysis,  and  to  subdivide  them,  according  to  their 
more  prominent  symptoms,  into  a  spinal,  and  a  cerebral  or 
a  cerebro-spinal  variety. 

After  adopting  a  subdivision  of  this  kind,  the  pathology 
of  each  type  will  be  more  readily  made  out ;  and  it  is  quite 
probable  that  the  various  types  will  be  found  to  represent 
a  mere  difference  in  the  topographical  distribution  of  le- 
sions and  symptoms,  while  the  morbid  process,  an  arrest  of 
development,  or  an  early  degeneration,  is  common  to  all. 

The  spinal  type  of  hereditary  spastic  paralysis  is  well 
illustrated  by  Newmark's  cases.  In  view  of  the  impor- 
tance of  the  subject  a  short  summary  of  a  few  of  his  cases 
will  be  given. 

A  girl,  aged  fifteen,  tall  and  intelligent ;  normal  birth  and  labor  ;  no  con- 
vulsions. Did  not  kick  with  the  legs  as  other  children  do  in  bed.  Began'  to 
walk  at  eighteen  months.  Gait  peculiar  from  the  first,  and  always  remained 
typical  of  spastic  paraplegia.  Lower  extremities  flexed  at  the  hips  and 
knees ;  adduction  of  thighs  and  pes  equinus.  No  wasting  of  muscles,  which 
react  well  to  both  currents.  Knee-jerks  and  adductor  reflex  exaggerated  ; 
no  ankle  clonus  (probably  on  account  of  extreme  contracture).  No  disturb- 
ance in  the  upper  extremities,  but  all  tendon  reflexes  exaggerated  in  them. 
Jaw-jerk  present.  Intelligence,  sensations,  and  sphincters  intact.  No  nys- 
tagmus, no  strabismus.  Girl  is  able  to  use  a  tricycle.  Speech  is  normal. 
A  brother,  aged  five,  exhibits  very  similar  symptoms.  No  history  of  syphilis 
in  the  family.  There  were  no  other  cases  of  similar  nature  in  the  family,  ex- 
cept one  of  spastic  diplegia  in  a  first  cousin ;  but  this  diplegia  was  evidently 
the  result  of  difficult  labor. 

In  the  second  family  the  father  is  said  to  be  a  tall,  healthy  man  of  thirty- 
eight,  who  denies  syphilitic  infection  or  alcoholic  habit.  Has  never  known 
of  any  affection  similar  to  that  observed  among  his  children.  His  reflexes 
are  normal.  Mother  of  children,  aged  thirty-seven,  has  always  been  healthy. 
Her  knee-jerks  and  Achilles  tendon  reflex  quite  active.  Lively  reflexes  in 
the  upper  extremities  and  jaw-jerk  distinct ;  no  ankle  clonus.  No  blood  re- 
lationship between  man  and  wife.  They  have  had  eleven  children,  eight 
of  whom  are  living.  The  oldest  living  child  is  a  boy,  aged  sixteen,  who 
was  well  until  about  a  year  and  a  half  before  he  was  examined.     At  that 


HEREDITARY  DISEASES   OE   THE   SPINAL    CORD.         347 

time  a  stiffness  appeared  in  the  legs,  especially  on  rising  in  the  morning. 
His  condition  gradually  developed  into  a  mild  form  of  spastic  paraplegia, 
with  typical  gait  and  position  of  the  extremities.  Knee-jerks  much  exag- 
gerated ;  no  patellar  clonus  nor  ankle  clonus.  Plantar,  cremasteric,  and 
abdominal  reflexes  very  lively.  Abdominal  muscles  said  to  be  rigid.  Up- 
per limbs  normal ;  reflexes  much  increased.  Jaw-jerk  well  marked.  In  a 
second  brother,  aged  fourteen,  the  symptoms  had  become  more  pronounced, 
and  had  set  in  at  the  age  of  seven  and  a  half  years.  The  legs  were  stiff  and 
walking  was  difficult ;  had  been  compelled  to  use  crutches.  There  were  con- 
tractures in  the  knee-joints  ;  there  was  adductor  spasm  and  pes  equinus, 
furthermore,  an  exaggeration  of  the  knee-jerks.  Ankle  clonus  was  present, 
and  the  plantar  and  cremasteric  reflexes  were  also  very  lively.  A  third 
brother  developed  exactly  the  same  symptoms  at  the  age  of  nine  years,  after 
typho-malarial  fever.  Though  these  three  cases  are  the  most  pronounced 
ones  in  the  family,  one  sister  exhibits  increased  reflexes  ;  a  boy,  eight  years 
of  age,  is  said  to  be  stiff  in  the  knees,  with  exaggeration  of  the  patellar  re- 
flex ;  another  child,  aged  six,  is  said  to  have  a  lazy,  dragging  walk,  with  ex- 
cessive increase  of  knee-jerks ;  a  child,  aged  three  and  one  half,  has  active 
knee-jerk  and  jaw-jerk. 

That  there  is  a  tendency  to  spastic  paralysis  in  this 
family  there  is  no  doubt ;  but  the  fact  that  instruments  were 
used  at  the  birth  of  several  children,  including  the  one  whose 
symptoms  were  most  pronounced,  makes  it  probable  that 
"  heredity  "  alone  was  not  responsible  for  the  multiplicity 
of  cases.  In  a  more  recent  publication  (1904)  Newmark 
reports  upon  the  development  of  the  children  referred  to  in 
his  earlier  articles. 

The  symptoms  of  this  spinal  type  (spastic  paraplegia, 
with  rigidity  or  contractures,  increased  reflexes)  are 
distinctly  due  to  interference  with  the  pyramidal  tracts 
in  the  lateral  columns  of  the  cord.  Many  years  ago 
Strumpell  described  the  cases  of  two  brothers,  who  pre- 
sented the  typical  features  of  a  spastic  spinal  palsy.  In 
one  of  these  cases  a  post-mortem  examination  was  made, 
by  which  it  was  determined  that  the  symptoms  were  due 
to  a  primary  systematic  degeneration  of  both  pyramidal 
tracts  in  the  lateral  columns,  together  with  a  slight  affec- 
tion of  the  cerebellar  tracts  and  the  columns  of  Goll.  More 
recently  Strumpell  has  described  another  case,  very  much 
like  the  other  two,  in  which  he  made  the  diagnosis  of  he- 
reditary spastic  palsy.  In  the  family  of  this  patient  the 
grandfather  was  said  to  have  suffered  from  a  palsy  of  the 


348  THE   NERVOUS  DISEASES   OF   CHILDREN. 

legs,  the  father  had  a  peculiar  gait,  and  people  who  knew 
both  the  father  and  the  present  patient  said  that  one  walked 
like  the  other.  Two  uncles  were  said  to  have  the  same  walk. 
The  mother  of  the  patient  and  other  brothers  and  sisters 
were  entirely  healthy.  The  patient  did  military  service 
from  his  twenty-first  to  his  thirty-third  year,  and  was  not 
inconvenienced  in  any  way,  except  that  he  occasionally 
noticed  a  peculiar  feeling  in  the  legs.  At  the  age  of  about 
thirty-five  his  first  symptoms  began.  His  legs  became  stiff 
and  he  soon  had  to  resort  to  a  stick  in  walking.  The  exam- 
ination of  the  patient  revealed  a  typical  spinal  spastic 
paralysis,  with  exaggeration  of  the  reflexes,  rigidity  of  the 
joints,  normal  sensation,  and  a  spastic  gait.  The  vesical  and 
rectal  reflexes  were  not  interfered  with.  All  the  symptoms 
steadily  increased,  but  no  new  ones  were  developed.  In 
addition  to  its  interest  as  a  form  of  hereditary  spinal  disease 
the  type  also  deserves  some  special  recognition  from  the 
fact  that,  from  first  to  last,  the  symptoms  have  been  such 
as  are  due  exclusively  to  disease  of  the  lateral  columns. 
Strumpell  refers  to  Bernhardt's  patients,  and  claims  that 
they  are  entirely  identical  with  those  described  by  him. 
Four  out  of  six  brothers  presented  the  phenomena  of  spastic 
spinal  palsy  ;  three  of  these  four  developed  their  symptoms 
at  the  age  of  thirty,  and  all  the  morbid  signs  progressed  in  a 
remarkably  slow  fashion.  I  can  agree  with  Strumpell  in 
believing  that  these  diseases  are  entirely  similar  to  those  de- 
scribed by  him,  although  in  one  case  of  Bernhardt  the  symp- 
toms tended  toward  a  cerebro-spinal  rather  than  a  purely 
spinal  type.  The  forms  described  by  Tooth  and  Philip 
should  be  placed  in  the  same  category. 

Spastic  spinal  paralysis  (spastic  paraplegia)  occurs  in  children  of  families 
in  which  there  is  no  history  of  a  similar  affection  in  the  same  or  past  genera- 
tions. In  February,  1893,  a  girl,  four  years  of  age,  was  brought  to  my  clinic, 
who  was  afflicted  with  a  typical  spastic  paraplegia  ;  paresis  and  rigidity  of 
both  lower  extremities  ;  very  rigid  gait,  increased  knee-jerks  on  both  sides, 
slight  double  ankle  clonus — these  reflexes  being  a  little  more  marked  on  the 
right  side  than  on  the  left  side  ;  increased  reflexes  in  the  upper  extremities,  but 
no  weakness.  Electrical  reaction  of  all  muscles  and  nerves  normal ;  no  dis- 
turbances of  sensation ;  no  impairment  of  vesical  and  rectal  reflexes ;  no 
nystagmus.  Intelligence  good.  The  birth  of  the  child  (first-born)  had  been 
entirely  normal  and  at  full  term  ;  no  instruments  were  used  ;  the  child  was 


HEREDITARY  DISEASES   OF  THE    SPINAL    CORD.        349 

supposed  to  be  perfectly  well,  except  that  it  did  not  begin  to  walk  until  it  was 
three  years  old,  and  its  walk  was  unusually  stiff  and  awkward  ;  it  dragged  its 
feet.  Three  younger  children  are  perfectly  healthy.  Excepting  the  early  age 
of  onset  there  is  no  distinction  in  clinical  symptoms  between  such  cases  as 
these  and  the  spastic  spinal  paralysis  of  the  adult. 

Morbid  Anatomy. — In  one  of  Newmark's  cases  there 
was  a  distinct  degeneration  of  the  pyramidal  tracts,  most 
marked  in  the  lumbar  portion  of  the  cord;  a  degeneration 
of  the  posterior  columns,  most  marked  in  the  upper  dorsal 
region  ;  also  an  involvement  of  the  columns  of  Clarke — in 
short,  a  combined  degeneration  not  unlike  the  findings  in 
Striimpell's  cases  and  in  Friedreich's  ataxia.  In  view  of 
Striimpell's  investigations  there  is  no  longer  any  doubt 
about  the  occurrence  of  a  primary  degeneration  of  the 
lateral  columns  developing  later  in  life,  but  due  in  all  proba- 
bility to  abnormal  congenital  conditions.  There  is  some 
reason,  therefore,  to  believe  that  the  question  of  the  occur- 
rence of  a  primary  lateral  sclerosis  will  be  affirmatively  an- 
swered through  these  studies  on  hereditary  spastic  paralysis. 

Diagnosis. — The  diagnosis  of  these  diseases  need  not 
be  given  in  further  detail.  The  history  of  a  case  exhibiting 
the  symptoms  of  spastic  paraplegia  of  the  lower  extremities, 
with  increase  of  the  reflexes,  with  rigidities  and  contrac- 
tures, without  involvement  of  the  vesical  or  rectal  reflexes, 
without  atrophy,  without  disturbances  of  speech  and  nys- 
tagmus, would  be  sufficient  to  place  the  case  in  this  cate- 
gory, provided  the  hereditary  character  of  the  affection 
could  be  established.  The  disease  should  be  carefully  dif- 
ferentiated from  the  spastic  cerebral  palsies  of  childhood.* 
The  diplegias  and  paraplegias  occurring  as  a  result  of  diffi- 

*  In  an  article  on  Hereditary  Spastic  Paralysis,  Erb  stated  that  some  of  the  con- 
genital diplegias  and  paraplegias  which  Freud,  myself,  and  others  have  attributed  to 
cerebral  lesions  may  at  times  be  due  to  spinal  lesions.  I  am  willing  to  concede  the 
possibility  of  this  ;  but  I  think  that  such  spinal  affections  are,  after  all,  rare  ;  and  there 
is  the  one  fact,  to  which  I  have  alluded  in  various  writings,  that  in  forty-five  per  cent, 
of  all  the  cases  of  infantile  spastic  paraplegia  there  is  marked  idiocy.  This  association 
militates  against  the  diagnosis  of  a  purely  spinal  disease.  I  have  reviewed  the  his- 
tories of  all  my  patients  with  supposed  cerebral  paraplegia  and  can  find  none  which  I 
am  willing  to  attribute  to  a  spinal  lesion.  The  child  reported  on  page  348,  though  suf- 
fering from  paraplegia,  was  recognized  as  probably  of  spinal  origin  ;  and  such  an 
origin  may  be  suspected  in  types  of  spastic  paraplegia,  without  idiocy  coming  on 
several  years  after  a  normal  birth. 


350  THE  NERVOUS  DISEASES   OF   CHILDREN. 

culty  during  labor  (Little's  disease),  would  be  most  easily 
confounded  with  this  hereditary  form.  A  careful  inquiry 
into  the  history  of  labor,  the  determination  of  the  exact  age 
at  which  the  trouble  began,  the  frequent  occurrence  of  con- 
vulsions and  of  defective  mental  development,  in  birth 
palsies,  will  help  to  differentiate  between  these  two  sets  of 
cases.  If  spastic  paraplegia  is  developed  in  a  child,  or  in  a 
youth,  that  has  not  been  preceded  by  convulsions,  or  is  not 
associated  with  defective  mental  development,  the  case 
would  come  more  properly  under  the  head  of  hereditary 
spastic  paraplegia,  and  we  must  remember  that  such  cases 
may  occur  without  any  history  of  similar  disease  in  the  same 
or  preceding  generations.* 

The  prognosis  of  hereditary  spastic  spinal  paralysis  is 
a  grave  one  as  regards  recovery  from  the  disease,  but  not 
unfavorable  as  regards  the  duration  of  life,  for  the  majority 
of  patients  thus  far  examined  have  been  well  advanced  in 
years.  In  the  way  of  treatment,  nothing  can  be  attempted 
except  to  apply  the  usual  methods  of  massage  and  elec- 
tricity, or  possibly  to  attempt  to  improve  contractured 
limbs  by  various  tenotomies  and  by  transplantation  of 
tendons. 

CONGENITAL   SPASTIC   PARAPLEGIA.      (LITTLE'S   DISEASE.) 

In  this  connection  brief  reference  must  be  made  to  an  affection  which  is 
generally  spoken  of  as  "  Little's  Disease."  It  refers  to  a  condition  of  spastic 
rigidity  of  the  limbs  with  paralysis  which  is  observed  in  the  earliest  months 
and  years  of  life,  and  is  almost  invariably  due  either  to  defective  development 
or  to  injuries  received  during  protracted  labor.  Van  Gehuchten,  without 
sufficient  justification,  restricts  the  term  to  the  disease  occurring  in  children 
born  before  full  term.  As  the  present  author  showed  in  1897,  so  many 
different  conditions  have  been  collated  under  the  term  Little  s  Disease  that 
much  confusion  has  resulted.  It  will  be  well,  therefore,  to  quote  from 
Little's  original  paper   in    "  The  Transactions   of   the   London   Obstetrical 

*  Marie  and  some  other  French  authors  deny  the  existence  of  a  primary  spinal 
spastic  paralysis  in  the  adult,  and  believe  that  if  such  a  condition  does  exist,  it  is  to  be 
traced  back  to  the  early  years  of  life.  They  describe  as  "  Tabes  dorsal  spasmodique  " 
of  children,  a  group  of  symptoms  which  German  and  American  authors  have  included 
under  the  term,  Congenital  Spastic  Diplegia  and  Paraplegia.  The  French  view  would 
take  all  of  these  cases  out  of  the  category  of  cerebral  diseases,  and  for  this  there  is  as 
yet  no  warrant.  Marie  (p.  101)  states  that  "Tabes  dorsal  spasmodique"  is  never 
hereditary  ;  and  attributes  the  condition  to  defective  development  of  the  pyramidal 
tract.     This  tract  is  surely  a  cerebro-spinal  affair. 


HEREDITARY  DISEASES   OF  THE  SPINAL    CORD.        35 1 

Society,"  Vol.  3,  1862,111  which  he  says:  "I  showed  that  premature  birth, 
difficult  labors,  mechanical  injuries  during  parturition  to  head  and  neck 
where  life  has  been  saved,  convulsions  following  the  act  of  birth,  were  apt  to 
be  succeeded  by  a  determinate  affection  of  the  limbs  of  the  child  which  I 
designated,  spastic  rigidity  of  the  limbs  of  new-born  children,  spastic  rigidity 
from  asphyxia  neonatorum  and  assimilated  it  to  the  trismus  nascentium 
and  the  universal  spastic  rigidity  sometimes  produced  at  later  periods  of 
existence." 

He  speaks  of  spastic  rigidity  being  sometimes  associated  with  paralytic 
contraction.  He  evidently  had  a  paralytic  condition  in  mind,  and  in  that 
respect  various  recent  writers  who  seem  to  deny  the  existence  of  paralysis  in 
these  cases  and  wish  to  speak  of  them  only  as  spastic  rigidities,  are  in  the 
wrong. 

In  association  with  Dr.  Peterson  I  showed  many  years  ago  that  the 
frequent  occurrence  of  mental  defect  with  congenital  spastic  rigidity  and 
paralysis  was  due  to  the  various  cerebral  lesions  and  accidents  which  are 
responsible  for  other  cerebral  palsies  of  children.  The  symptoms  are,  as  a 
rule,  limited  to  the  lower  extremities,  although  on  examination  some  slight 
difficulty  in  the  upper  extremities  may  frequently  be  noted.  Cross-legged 
position  and  cross-legged  gait  are  typical  symptoms  of  this  trouble.  There 
is,  of  course,  close  resemblance  to  disease  involving  the  lateral  columns  of 
the  cord,  and  in  this  respect  the  cases  must  be  carefully  differentiated  from 
primary  or  hereditary  spastic  paralysis  and  from  syphilitic  spinal  paralysis. 

Recent  observations  of  Dejerine  and  of  Spiller  have  shown  that  con- 
genital spastic  paraplegia  may  be  due  to  changes  in  the  lateral  columns 
of  the  cord  only,  without  any  cerebral  disease,  but  in  spite  of  these  two 
trustworthy  findings,  the  present  writer  still  believes  that  in  most  instances 
the  conditions  which  could  reasonably  be  designated  as  Little's  Disease,  are 
to  be  attributed  to  defective  development  of,  or  to  lesions  in,  the  brain. 
Further  information  on  this  subject  can  be  gathered  from  the  chapter  on 
infantile  cerebral  palsies.  It  will  be  recognized  that  if  the  term  Little's 
Disease  is  to  be  retained  at  all,  it  should  be  applied  only  to  the  cases  of 
spastic  paraplegia,  or  of  spastic  diplegia  (complete  or  incomplete),  due  to 
premature  birth  or  to  difficulties  during  parturition. 


CHAPTER   XXI. 

PROGRESSIVE  MUSCULAR  ATROPHIES. 

In  this  chapter  we  intend  to  discuss  all  those  diseases 
which  are  characterized  by  a  progressive  weakness  and 
atrophy  of  certain  groups  of  muscles.  We  have  nothing 
to  do  with  muscular  atrophy,  whether  progressive  or  not, 
which  follows  after  acute  disease  of  the  brain,  of  the  spinal 
cord,  or  of  the  peripheral  nerves.  The  term  "progressive 
muscular  atrophy  "  was  formerly  given  to  a  single  type  of 
disease  with  which  we  shall  become  more  intimately  ac- 
quainted, and  for  a  time  all  cases  resembling  this  one  type 
were  designated  in  the  same  way.  It  became  evident,  how- 
ever, that  this  one  term  was  altogether  too  general.  While 
it  was  a  convenient  clinical  designation  for  an  entire  group 
of  diseases,  it  did  not  sufficiently  describe  a  number  of 
other  forms  which  were  closely  allied  to  the  chief  type. 
In  order  to  avoid  confusion,  it  would  be  well  if  we  could 
dismiss  the  term  progressive  muscular  atrophy  altogether, 
for  in  many  of  the  cases  hypertrophy  as  well  as  atrophy  is 
present  for  a  long  period  of  time.  The  word  dystrophy, 
which  might  be  used  to  designate  both  conditions,  has,  un- 
fortunately, been  restricted  to  the  cases  of  primary  or  idio- 
pathic muscular  wasting ;  we  cannot,  therefore,  apply  it  to 
the  spinal  forms.  Moreover,  a  number  of  diseases  were 
included  under  the  heading  of  progressive  muscular  atrophy 
which  we  now  recognize  to  have  been  cases  of  amyotrophic 
lateral  sclerosis,  of  syringomyelia,  and  possibly  of  spinal 
syphilis. 

The  chief  question  at  the  present  time  regarding  the 
various  forms  of  progressive  muscular  atrophy  is,  whether 
in  a  given  case  the  disease  is  of  spinal  or  muscular  origin. 


PR  O  G  RES  SI  I  rE    M  USC  ULA  R   A  TR  OP  II J  h  .'/. 


353 


Amyotrophy 


Neural  Atrophy 


Those    forms   of    progressive   muscular  atrophy   due   to  a 

spinal  lesion  we  call  amyotrophies,  and  to  those  due  to  disease 
of  the  muscular  system  alone,  we  give  the  name  myopathies. 
We  shall  see  that  there  is  some  reason,  too,  to  (  onstitute  a 
third  type,  which  we  might  designate  as  a  neural  form  of 
progressive  muscular  atrophy.     (Fig.  83.) 

The  study  of  this  entire  subject  began  many  years  ago 
with  the  establishment  of  two  distinct  diseases — the  first  was 
the  typical  "progressive  muscu- 
lar atrophy,"  as  described  by 
Aran  and  Duchenne ;  the  sec- 
ond, pseudo-hypertrophic  mus- 
cular paralysis.  Since  that 
time  at  least  six  different  forms 
of  progressive  muscular  wast- 
ing have  been  described,  and 
the  attempt  has  been  made  in 
each  case  to  prove  the  relation 
of  the  special  form,  either  to 
the  Aran-Duchenne  type,  or  to 
the  type  of  muscular  pseudo- 
hypertrophy. The  Aran-Du- 
chenne type  has  become  the 
chief  exponent  of  progressive 
amyotrophies  ;  while  muscular 
pseudo-hypertrophy  has  been 
considered  the  most  pro- 
nounced form  of  primary  myo- 
pathies. The  various  types  of 
progressive  muscular  disease 
have  been  established  very  largely  in  accordance  with 
the  mere  topographical  distribution  of  atrophy  or  hyper- 
trophy. Though  convenient  for  clinical  designation,  such 
a  distinction  is  not  sufficient  for  a  rational  classification 
of  these  various  diseases.  It  must  be  our  aim  to  find  the 
cardinal  S)^mptoms  which  will  help  us  to  differentiate  at 
once,  and  easily,  between  those  cases  of  progressive  mus- 
cular disease  due  to  spinal-cord  lesions  and  those  primary 
dystrophies,  which  represent  a  disease  of  the  muscular 
system. 


Myopathy 


Fig.  83 — A  Diagram  designed  to  show 
the  Site  of  the  Morbid  Lesion  in  the 
Several  Groups  of  Progressive  MuS' 
cular  Atrophy. 


354  THE  NERVOUS  DISEASES   OF    CHILDREN. 

The  following  are  the  cardinal  symptoms  present  in  the  majority  of  cases 
belonging  to  the  two  large  groups  of  cases  : 

Progressive  Amyotrophies.  Progressive  Myopathies. 

Onset   late   in   life  ;    rarely   in  early  Onset  in  early  life. 

childhood. 

Not  hereditary,  as  a  rule.  Generally  hereditary  (family  trouble). 

Wasting  first  in  the  upper  extremi-  Wasting  or  hypertrophy   begins   in 

ties  (leg  type  rare).  the  lower  extremities. 

Hypertrophy  does  not  occur.  Hypertrophy  frequent. 

Fibrillary  twitchings.  No  fibrillary  twitchings. 

Reaction  of  degeneration  often  pres-  Reaction  of  degeneration  rare  (quan- 

ent  in  affected  muscles.  titative,   not   qualitative,   electrical 

changes). 

These  points  of  differential  diagnosis  will  hold  good  in  the  majority  of  cases. 
Werdnig,  Hoffmann,  and  others  have  reported  cases  of  spinal  progressive 
muscular  disease  due  to  hereditary  or  family  influences.  Fibrillary  twitch- 
ing has  been  seen  in  some  cases  that  appeared  to  be  typical  myopathies, 
and  the  electrical  reactions  have  been  found  considerably  altered  in  similar 
cases,  so  that  of  all  these  cardinal  symptoms  there  are,  after  all,  only  a  very 
few  which  are  invariably  present  in  one  or  the  other  form  of  progressive  mus- 
cular disease,  and  it  is  wiser,  therefore,  to  be  guided  by  the  general  agree- 
ment of  symptoms  rather  than  by  any  one  single  symptom.*  This  confusion 
of  types  of  disease  and  of  symptoms,  need  not  cause  surprise,  if  we  remem- 
ber that  the  ganglion  cells  of  the  spinal  cord,  the  peripheral  nerves,  and  the 
muscles,  constitute  a  physiological  unit  (Neuron). 

With  these  prefatory  remarks  we  may  proceed  to  the 
consideration  of  that  class  of  cases  which  for  a  very  long- 
time were  supposed  to  be  the  only  representatives  of  what 
was  formerly  called  "progressive  muscular  atrophy."  This 
is  a  spinal-cord  affection  which,  as  a  rule,  begins  late  in  life. 
It  might,  therefore,  be  considered  out  of  place  to  treat  of 
this  disease  in  a  work  on  the  nervous  diseases  of  children  ; 
but  the  entire  subject  of  muscular  diseases  cannot  be  prop- 
erly understood  unless  we  can  recognize  this  special  type, 
and,  furthermore,  cases  of  this  type  have  of  late  years  been 
described  by  Hoffmann  and  others,  in  chiLdren. 

*  The  hereditary  cases  reported  by  Werdnig  are  particularly  interesting  in  this  re- 
spect. 


PROGRESSIVE  MUSCULAR  ATROPHIES.  355 

PROGRESSIVE   AMYOTROPHY. — "PROGRESSIVE    MUSCULAR 
ATROPHY."      (TYPE,   ARAN-DUCHENNE.) 

This  form  begins,  in  the  majority  of  the  cases,  with  an 
atrophy  and  a  corresponding  weakness  in  the  small  muscles 
of  the  hand  (thenar  and  hypothenar).  The  atrophy  extends 
slowly  from  muscle  to  muscle  ("  atrophie  individuelle  "),  be- 
ginning as  a  rule  with  the  adductor  pollicis.  It  involves  by 
degrees  the  opponens  pollicis  and  the  deep  muscles  of  the 
thenar.  From  these  it  gradually  extends  to  the  muscles  of 
the  hypothenar,  the  interossei,  the  flexors  and  extensors  in 
the  forearm.  At  this  point  the  disease  may  remain  sta- 
tionary, or  it  may  spread  to  the  flexors  in  the  upper  arm,  to 
the  deltoid,  the  triceps,  and  finally  to  the  muscles  of  the 
trunk,  the  shoulders,  and  the  back.  Duchenne  recognized 
the  fact  that  the  atrophy  may,  in  exceptional  cases,  begin  in 
the  trunk,  in  the  shoulders,  or  in  the  legs.  Some  of  these 
would  now,  no  doubt,  be  considered  under  a  different  head- 
ing, but  I  have  myself  seen  cases  which  could  in  nowise 
be  distinguished  from  the  typical  Aran-Duchenne  disease 
which  began  in  the  muscles  of  the  thighs.  If  the  disease 
begins  in  the  upper  extremities,  the  legs  are,  as  a  rule,  not 
affected  until  very  late  in  the  course  of  the  disease ;  but 
there  are  exceptions  to  this  rule.  I  have  observed  several 
cases  in  which  the  atrophy  in  the  lower  extremities  began 
almost  simultaneously  with  that  in  the  upper  extremities. 

The  atrophied  muscles  in  this  form  exhibit  fibrillary  con- 
tractions, and,  as  a  rule,  present  marked  changes  in  electri- 
cal contractility.  These  changes  are  not  so  pronounced  as 
in  cases  of  acute  or  subacute  anterior  poliomyelitis,  and  yet. 
after  the  disease  has  lasted  for  a  considerable  length  of  time 
a  typical  reaction  of  degeneration  will  be  found  present  in 
most  of  the  wasted  muscles.  In  the  majority  of  cases  thus 
far  recorded  the  disease  has  not  been  distinctly  hereditary, 
although  series  of  such  cases  with  distinct  hereditary  ten- 
dencies have  been  published  by  Naunyn,  Eichhorst,  Ham- 
mond, Osier,  and  others.* 

*  The  famous  Weathersby  family,  reported  by  Hammond,  and  the  Farr  family,  of 
Vermont,  described  by  Osier,  may  possibly  represent  other  types  of  progressive 
muscular  atrophy,  which  we  shall  consider  later  on. 


356  THE  NERVOUS  DISEASES   OF  CHILDREN. 

As  the  disease  progresses  the  wasting  becomes  more  and 
more  extreme  ;  the  patient  is  no  longer  able  to  get  about, 
becomes  bedridden,  and  after  many  years  of  annoyance,  if 
not  of  suffering,  dies  of  some  intercurrent  disease,  or  from 
extension  of  the  process  upward  into  the  region  of  the  me- 
dulla, with  consequent  paralysis  of  the  vital  centres. 

The  symptoms  of  the  disease  were  well  described  by 
Duchenne,  and  very  little  has  been  added  since  his  day  to 
the  clinical  characterization  of  this  special  form.  But  a  very 
warm  discussion  was  waged  for  a  long  time  regarding  the 
origin  of  the  disease,  some  maintaining  its  spinal  origin, 
others  believing  it  to  be  a  peripheral  disease.  There  was 
some  reason  for  this  difference  of  opinion,  for  the  cases  upon 
which  the  older  authors  based  their  views  were  in  part  due 
to  spinal  disease,  and  in  part  due  to  nerve  or  muscular 
lesions.  The  microscopical  studies  of  Charcot  and  Joffroy, 
of  Lockhart  Clarke,  of  Hayem,  and  others,  proved  beyond 
a  doubt  that  this  special  form  of  progressive  muscular 
atrophy  was  due  to  changes  in  the  spinal  cord.  The  chief 
changes  found  are  these  :  A  sclerotic  and  pigmentary  atro- 
phy of  the  ganglion  cells  of  the  anterior  horns,  inflamma- 
tory changes  in  the  neuroglia,  increased  size  of  the  blood- 
vessels, and  proliferation  of  the  cellular  elements.  In  fresh 
preparations  granular  corpuscles  are  found,  and  according 
to  the  degree  and  stage  of  the  disease  the  anterior  horns 
may  be  very  much  reduced  in  all  diameters,  and  the  gan- 
glion cells  either  atrophied  or  entirely  lost.  The  anterior 
nerve-roots  are  affected  secondarily  to  the  lesion  of  the  gray 
substance.  The  nerve-fibres  are  not  all  destroyed,  a  num- 
ber of  them  remaining  intact.  Those  that  are  destroyed 
exhibit  the  appearances  of  simple  atrophy,  a  point  to  which 
Charcot  alludes  as  distinguishing  these  cases  from  infantile 
spinal  paralysis. 

According  to  these  pathological  findings  we  must  sup- 
pose that  an  inflammation  spreads  slowly  from  the  ganglion 
cells  of  the  anterior  horns  along  the  anterior  nerve-roots 
without  destroying  as  many  of  these  fibres  as  is  the  case  in 
infantile  poliomyelitis.  The  atrophic  changes  in  the  mus- 
cles are  the  direct  result  of  irritation,  which  begins  in  the 
cells  of  the  anterior  horns,  and  is  propagated  thence  through 


PROGRESSIVE  MUSCULAR  ATROPHIES.  357 

normal  or  only  half-wasted  nerve-roots,  to  the  peripheral 
muscular  fibre.  Positive  as  these  anatomical  findings  seem 
to  be,  it  is  somewhat  surprising  to  learn  how  few  reliable 
post-mortem  examinations  have  been  made  in  these  cases 
proving  the  correctness  of  these  views.  The  cases  of  Pier- 
rot-Troissier,  of  Strumpell  and  of  Hoffmann,  are  among 
the  few  which  have  been  so  carefully  examined  as  to  have 
placed  the  spinal  origin  of  this  special  type  of  progressive 
muscular  atrophy  beyond  question.  In  these  cases  the  an- 
terior gray  matter  was  the  only  part  affected,  and  alone  re- 
sponsible for  the  wide-spread  muscular  atrophy. 

There  is  no  need  in  this  treatise  to  give  a  typical  history 
of  a  case  of  spinal  progressive  muscular  atrophy,  as  it  oc- 
curs in  the  adult.  I  have  stated  above  that  these  cases  are, 
as  a  rule,  not  hereditary,  and  upon  this  absence  of  the  factor 
of  inheritance  the  differential  diagnosis  was  formerly  fre- 
quently based  between  a  progressive  amyotrophy  and  a  pro- 
gressive myopathy.  But  this  point  of  differential  diagnosis 
has  been  rudely  shaken  by  the  interesting  articles  which 
Hoffmann  and  Werdnig  published  some  years  ago  concern- 
ing the  occurrence  of  chronic  spinal  muscular  atrophy  in 
children.  I  will  endeavor  to  summarize  one  of  Hoffmann's 
cases  of 

HEREDITARY   PROGRESSIVE    MUSCULAR    ATROPHY. 

A  girl,  four  years  of  age ;  the  birth  of  the  child  was  entirely  normal ; 
when  nine  months  of  age  was  able  to  stand ;  early  abnormal  development 
of  adipose  tissue.  Gradually  the  child  became  so  weak  that  it  could  not 
stand,  could  not  sit  upright  in  bed,  could  not  turn  around  without  assistance. 
For  a  long  time  it  was  able  to  move  its  feet  and  its  arms.  The  motor  dis- 
turbances increased  gradually,  and  the  child  lost  its  superfluous  fat  and  be- 
came thoroughly  emaciated,  particularly  in  the  trunk  and  the  extremities. 
The  face  remained  full.  The  sphincters  were  at  no  time  involved.  Mental 
development  was  good.  Speech  was  normal ;  no  convulsions  ;  no  strabismus ; 
no  difficulties  in  mastication  or  deglutition.  The  child  was  able  to  turn  its 
head,  but  could  not  lift  it  from  the  pillow.  There  was  no  evidence  of  any 
hypertrophy  or  pseudo-hypertrophy  in  any  of  the  muscles,  but  there  was  pa- 
resis and  atrophy  of  the  deep  muscles  of  the  neck,  of  the  sterno-cleido-mas- 
toid,  of  the  trapezius,  of  most  of  the  shoulder-muscles,  of  the  latissimus  dorsi, 
of  the  serrati,  the  pectoral  and  deltoids,  and  of  the  flexor  muscles  in  the  arm. 
The  biceps  and  brachialis  anticus  were  very  thin,  the  supinator  longus  a  little 
stronger  than  these.     The  triceps  was  thin  and  weak.     The  extensors  and 


35§  THE  AERVOUS  DISEASES   OF   CHILDREN. 

flexors  in  the  forearm  were  also  atrophic  and  paretic.  The  thenar  and  hypo- 
thenar  eminences  were  thin,  flaccid,  and  weak,  as  were  also  the  individual  in- 
terossei.  The  paralysis  was  in  proportion  to  the  atrophy  of  these  muscles. 
The  tendon  reflexes  were  entirely  wanting  in  the  upper  extremities.  The 
mechanical  excitability  of  the  muscles  was  diminished.  The  nerve-trunks 
were  neither  thickened  nor  sensitive  on  pressure.  The  paresis  and  atrophy 
of  both  upper  extremities  were  entirely  symmetrical.  There  were  no  fibrillary 
movements.  There  were  no  trophic  or  vasomotor  disturbances  of  the  skin 
in  the  upper  extremities.  Sensation  was  entirely  normal.  There  was  marked 
diminution  of  electrical  excitability  in  the  median  and  ulnar  nerves,  and  com- 
plete reaction  of  degeneration  in  the  biceps. 

The  muscles  of  the  back  and  abdomen  were  very  paretic,  the  long  mus- 
cles of  the  spine  much  diminished  in  volume  and  power.  There  was  lor- 
dosis of  the  lumbar  region  of  the  spinal  column.  The  gluteal  muscles,  and 
all  the  muscles  of  the  thigh,  were  very  atrophic  and  almost  completely  para- 
lyzed. The  muscles  of  the  leg  were  also  atrophic  and  paretic.  Movements 
of  the  toes  tolerably  good.  A  progressive  diminution  in  the  volume  of  the 
calf  and  thigh  muscles  was  noted  at  periods  six  months  apart.  No  indica- 
tion of  any  deep  reflexes  in  the  lower  extremities.  The  faradic  excitability 
of  the  nerves  in  the  lower  extremities  was  distinctly  diminished.  No  sensory 
disturbances,  no  fibrillary  or  fascicular  or  choreic  movements  of  muscles. 
The  paralysis  was  symmetrical  and  flaccid.  The  joints  were  like  those  of 
infantile  spinal  palsy.  The  child  died  of  an  intercurrent  pulmonary  trouble 
about  one  year  after  the  first  examination. 

The  report  of  the  autopsy  included  the  following  points  :  The  lumbar 
portion  of  the  spinal  cord  less  in  volume  than  under  normal  conditions. 
Very  marked  atrophy  of  the  anterior  spinal-cord  roots  throughout  the  entire 
spinal  cord  as  high  up  as  the  medulla.  On  microscopical  examination  the 
chief  changes  found  were  in  the  anterior  gray  matter  and  in  the  anterior 
nerve-roots,  from  the  lower  portion  of  the  medulla  through  the  whole  spinal 
cord.  There  was  a  distinct  atrophy  and  diminution  in  number  of  the  gan- 
glion cells  of  the  anterior  horns  throughout  the  entire  spinal  cord.  This  was 
more  marked  in  the  lumbar  than  in  the  cervical  portion.  There  was  also 
very  marked  atrophy  of  the  anterior  roots  and  a  similar  affection  in  the 
peripheral  nerves  and  the  nerve-filaments  in  the  muscles,  as  well  as  a  very 
marked  atrophy  of  the  muscles  supplied  by  these  nerves.  In  addition  to 
these  chief  changes  there  were  also  slight  and  symmetrical  changes  in 
the  motor  tracts  of  the  spinal  cord,  particularly  in  the  crossed  pyramidal 
tracts  and  in  the  lateral  columns  as  well  as  in  the  direct  pyramidal  tracts. 
Medulla  oblongata  was  not  involved. 

This  case  is  so  similar,  from  a  clinical  and  anatomical  point  of  view,  to 
the  typical  cases  of  the  Aran-Duchenne  type  that  the  close  relationship  be- 
tween these  forms  cannot  be  doubted.  It  remains  to  add  that  an  entirely 
similar  affection  was  reported  in  the  case  of  a  brother  of  the  first  child,  in 
whom  the  disease  began  at  about  the  same  age  and  behaved  in  very  much 
the  same  way.  The  parents  of  these  two  children  have  raised  a  family  of 
fifteen  ;  several  of  these  have  died  of  convulsions,  others  are  afflicted  with 


PROGRESSIVE  MUSCULAR   ATROPHIES.  359 

distinct  lipomatosis,  and  the  parents  invariably  accepted  the  occurrence  of 
this  excessive  accumulation  of  fat  as  an  evil  omen. 

Abortive  and  hereditary  forms  of  progressive  muscular  atrophy  of  the 
spinal  type  have  come  under  my  notice.  The  cases  are  those  of  a  physician, 
living  in  Canada,  and  his  daughter,  about  twelve  years  of  age ;  in  both  of 
them  there  was  distinct  atrophy  of  the  interossei  of  both  hands,  flattening  of 
the  thenar,  and  slight  extension  of  the  atrophy  to  muscles  of  the  forearm. 
"  Weak  hands  "  have  been  characteristic  of  the  family,  but  the  disease  does 
not  appear  seriously  to  invade  other  parts.  The  father  (physician)  has  had 
this  trouble  for  years,  and  has  been  compelled  to  give  up  surgical  work. 


PROGRESSIVE  NEURAL  MUSCULAR  ATROPHY — PROGRESSIVE 
NEUROTIC  MUSCULAR  ATROPHY — THE  PERONEAL  FORM, 
OR   LEG  TYPE,   OF   PROGRESSIVE   MUSCULAR    ATROPHY.* 

In  this  form  of  progressive  muscular  wasting  the  dis- 
ease begins,  in  the  majority  of  cases,  in  the  lower  extrem- 
ities. At  first  the  extensor  muscles  of  the  toes  show  a 
slight  weakness.  The  small  muscles  of  the  feet  may  be- 
come involved,  and  then  the  atrophy  spreads  very  much 
after  the  fashion  of  the  spreading  of  the  atrophy  in  the 
spinal  cases,  from  muscle  to  muscle,  until  the  entire  leg  is 
considerably  atrophied  and  weakened.  As  a  result  of  this 
weakening,  deformities  of  the  foot  may  arise  ;  pes  equinus 
or  pes  equino-varus  is  a  frequent  result.  In  other  cases  a 
distinct  club-foot  is  developed,  and  inasmuch  as  the  affec- 
tion may  spread  quite  rapidly  from  one  side  to  the  other, 
a  progressive  form  of  wasting  in  both  lower  extremities, 
including  possibly  the  development  of  double  club-foot,  is 
extremely  suggestive  of  this  "  leg  type "  of  progressive 
muscular  atrophy.  The  disease,  as  Hoffmann  has  shown,  in 
rare  instances  attacks  the  upper  extremities  first  and  then 
involves  the  lower.  Hoffmann  has  objected  to  the  use  of 
the  designation  "  leg  type,"  but  since  in  this  form  the 
legs  are  involved  at  a  very  early  stage,  whethsr  the  disease 
begins  in  them  or  in  the  upper  extremities,  it  seems  proper 
for  the  present  to  retain  this  designation.  The  atrophy  in 
the  upper  extremities  may  involve  the  small  muscles  of  the 
hand,  the  extensor  and  flexor  muscles  of  the  forearm  or  the 

*  This  disease  is  also  known  as  the  type  of  Charcot-Marie-Tooth.  Hoffmann  sug- 
gested the  term  "  progressive  neurotic,"  Bernhardt,  the  term  "progressive  neural," 
muscular  atrophy.  , 


360 


THE   NERVOUS  DISEASES    OF   CHILDREN. 


arm,  and  may  cause  a  wasting  of  the  muscles  about  the 
shoulder-girdle.  I  have  seen  the  infra-  and  supra-spinati 
especially  wasted  in  several  of  these  cases.  The  atrophy 
in  the  upper  extremities  is,  as  a  rule,  not  so  distinct  nor  so 
early  a  symptom  as  in  the  cases  of  the  Aran-Duchenne  type. 


Fig.  84. —  Two  Brothers  afflicted  with  the  Peroneal  Form  of   Progressive  Muscular 
Atrophy,  Eight  Months  and  One  Year  respectively  after  First  Operation. 


Sensory  changes  are  generally  present,  and  serve  as  an 
important  point  of  differentiation  between  this  special  form 
of  atrophy  and  a  spinal  amyotrophy.  The  various  forms 
of  sensation  may  be  slightly  altered,  or,  in  some  cases,  tac- 
tile sensation  and  temperature  sense  may  remain  normal, 
while  the  pain  sense  may  be  more  distinctly  involved.  Par- 
gesthesias  may  be  present  in  addition  to  the  objective 
changes  in  sensation.  The  reflexes  in  the  lower  extremities 
are  either  diminished  or  lost ;  the  exact  state  of  the  reflexes 


PROGRESSIVE  MUSCULAR  ATROPHIES.  36 1 

depending  somewhat  upon  the  stage  of  the  disease  at  the 
time  the  patient  is  examined.  The  electrical  reactions  in 
the  atrophied  muscles  are,  as  a  rule,  altered.  The  changes 
are  not  so  extreme  as  in  the  cases  of  spinal  amyotrophy, 
nor  are  they  as  mild  as  in  the  primary  muscular  dystrophies. 
The  reactions  are  diminished  quantitatively,  and  altered 
also  as  regards  the  quality  of  contractions.  A  case  has  not 
yet  been  reported  in  which  the  muscles  of  the  face  were 
involved,  and  there  were  no  changes  in  sensation,  and  none 
in  electrical  reactions  in  any  of  the  muscles  or  nerves 
of  the  neck  and  head. 

The  symptomatology  of  this  rare  form  will  be  best  elucidated  by  an 
extract  from  the  account  given  of  the  disease  as  it  occurred  in  two  brothers 
which  was  reported  in  a  paper  published  by  the  author  in  the  year  1890. 
These  cases  are  all  the  more  interesting  as  they  are  the  only  ones,  to  my 
knowledge,  which  for  a  time  were  successfully  treated  by  surgical  measures. 

The  family  history  is  very  meagre.  The  father,  a  Bavarian,  is  dead ; 
cause  of  death  unknown.  Mother,  living  and  healthy,  thirty-two  years  of 
age.  The  two  patients  were  the  only  children.  Both  boys  were  born  healthy. 
Each  showed  disturbances  in  the  use  of  the  legs  at  a  very  early  day,  and  at 
the  age  of  five  years  both  had  acquired  double  club-foot. 

When  the  younger  brother  was  first  admitted  to  the  hospital,  in  1887,  he 
was  compelled  to  use  crutches.  According  to  the  hospital  records  there 
was  marked  shortening  of  the  Achilles  tendon  and  plantar  fascia  of  both  feet. 
Foot  arched  (pes  cavus)  ;  when  at  rest  inner  side  does  not  touch  the  floor. 

Measurements  r  Right  calf,  7  inches  ;  left  calf,  7  inches.  Dr.  Gibney 
performed  double  achillotomy.  Separation  of  ends  ij4  inch  on  the  left 
and  almost  the  same  on  the  right  side.  Feet  were  flexed  dorsally  to  about 
eighty  degrees,  and  plaster-of-Paris  splints  were  supplied.  He  was  dis- 
charged four  months  after  the  operation,  with  a  note  that  the  patient  walks 
quite  well,  soles  flat  on  the  ground,  toes  slightly  inverted.  He  returned  to 
the  hospital  in  1888  with  paralytic  limp  and  with  a  position  of  the  feet  as  rep- 
resented in  the  accompanying  figure.  Double  achillotomy  was  again  per- 
formed, division  of  plantar  fasciae  was  made,  and  there  followed  application 
of  Thomas's  tarsoclast  and  plaster  splints  with  the  results  as  shown  in  Figs. 
ioi  and  102.  Two  months  after  the  operation  the  feet  were  in  typical  cal- 
caneus position,  when  using  his  shoes  without  apparatus  ;  standing  squarely 
on  the  soles  of  the  feet  he  shows  disposition  to  roll  feet  inward.  He  could 
voluntarily  flex  the  ankle-joint  a  little  beyond  ninety  degrees,  but  in  so  doing 
the  toes  were  hyperextended.  He  walks  very  much  as  children  do  with  a 
peroneal  type  of  poliomyelitis.     Marked  disposition  to  pes  varus. 

My  own  examination  elicited  the  following  points  :  The 
boy  was  of  stouter  and  shorter  stature  than  his  brother. 


362 


THE   NERVOUS  DISEASES   OF   CHILDREN. 


Intelligence  good.  His  broad  chest  and  fat  stomach  are  in 
curious  contrast  to  his  spindle-shaped  extremities.  Circum- 
ference of  chest,  26  inches.  Right  arm,  6j4  inches ;  left  arm, 
inches.     Right  forearm,  6y£  inches  ;  left  forearm,  65^ 


inches.  Grasp  of  both  hands  very  weak.  A  general  emaci- 
ation of  all  parts  of  upper  ex- 
tremities. Very  distinct  atro- 
phy of  infraspinatus.  In  the 
legs  general  atrophy  is  very 
well  marked.  The  right 
thigh,  four  inches  above  pa- 
tella, 1 1  inches  ;  left  thigh, 
io*^  inches.  Right  calf,  at 
greatest  circumference,  8 
inches  ;  left  calf,  8^  inches. 
The  boy  walks  with  a  slightly 
waddling  gait  and  has  great 
difficulty  in  climbing  the 
stairs.  He  can  raise  toes  slight- 
ly on  the  left  side,  less  well 
on  the  right  side.  Can  raise 
left  leg  on  tiptoe,  but  cannot 
do  this  with  the  right  leg.  In 
attempting  to  raise  the  whole 
body  on  tiptoe,  falls  forward. 
Sensation :  Tactile  sensation 
normal  as  determined  by  cot- 
ton, pin  test,  and  the  writing 
of  numbers  on  the  skin.  Tem- 
perature sense  normal.  Pain 
sense  exaggerated.  Muscu- 
lar sense  normal.  Plantar  re- 
flexes present  and  knee-jerk  about  normal.  Slight  lividity 
of  legs,  not  so  marked,  however,  as  in  the  case  of  his 
brother. 


Fig.  85. 
(Same  patient  as  in  Fig.  86.) 


The  electrical  examination  :  In  the  upper  extremities  the  faradic  response 
in  the  median  and  ulnar  nerves  was  decidedly  diminished.  In  the  median 
nerve  first  KCC  with  13  MA;  ACC  not  at  20  MA.  Galvanic  current:  In 
the  right  leg  no  reactions  could  be  obtained  by  excitation  of  the  nerves  with 
currents  used.     In  the  extensor  hallucis  longus  the  first  KCC  and  ACC  were 


PROGRESSIVE   MUSCULAR   ATROPHIES. 


3r^3 


obtained  with  a  current  of  14  MA.  The  tibialis  anticus  did  not  respond  to 
currents  of  20  MA.  The  anterior  thigh  muscles  and  posterior  thigh  muscles 
respond  to  strong  currents  of  16  MA,  without  reversal  of  formula.  In  the 
left  leg,  the  extensor  hallucis,  first  KCC  with  16  MA  ;  first  ACC  with  18 
MA.  No  contractions  could  be  obtained  by  direct  excitation  of  the  tibialis 
anticus,  with  currents  up  to  20  MA  ;  on  excitation  of  the  extensor  digitorum 
communis  there  is  a  slight  movement 
of  the  small  toe. 

The  electrical  examination,  there- 
fore, shows  that  the  reaction  of  de- 
generation is  present  in  its  typical 
form  in  most  of  the  muscles  below 
the  patella,  the  galvanic  excitability 
of  the  peroneal  nerve  being  entirely 
lost.  It  also  shows  changes  in  elec- 
trical behavior  in  nerves  of  the  upper 
extremities,  since  the  responses  of 
the  ulnar  and  median  nerves  were 
markedly  diminished. 

Comparing  the  histories 
of  the  two  brothers,  it  was 
noted  that  they  resemble 
each  other  very  closely  as 
regards  the  first  appearance 
of  the  symptoms  and  the 
manner  in  which  the  disease 
spread  from  muscle  to  mus- 
cle ;  but  there  were  also 
certain  differences,  such  as 
the  more  marked  electrical 
changes  in  the  younger 
brother  and  the  greater  in- 
volvement of  the  upper  ex- 
tremities in  him,  than  in  the 
older  boy.  In  the  younger 
brother  the  disease  was  more 
fully  developed  in  every  re- 
spect than  in  his  older  broth- 
er ;  but  such  variations  as  occurred  were  within  a  reason- 
able limit  and  will  serve  to  show  to  what  extent  variations 
may  occur  in  persons  undoubtedly  suffering  from  the  same 
type  of  disease. 


Fig.  86.  —  The  Younger  of  the  Two 
Brothers  (see  Fig.  84)  after  Second 
Operation,  showing  Correction  of  De- 
formity of  Feet,  Marked  Atrophy  of 
Legs,  and  Incipient  Atrophy  of  Muscles 
above  Elbow  and  around  the  Shoulder 
Girdle. 


364  THE  NERVOUS  DISEASES   OF   CHILDREN. 

In  addition  to  the  symptoms  which  these  boys  have  exhibited,  it  is  inter- 
esting to  note  that  Vizioli  has  reported  the  occurrence  of  amaurosis  in  a 
similar  case,  due  to  an  optic-nerve  atrophy.  This  symptom  would  support 
the  argument  in  favor  of  the  nerve  origin  of  the  disease.  Furthermore  we 
may  insist  on  the  fact  that  hypertrophy  has  not  been  recorded  in  any  case, 
and  that  fibrillary  movements  seem  to  occur  in  some. 

Thomson  and  Bruce  have  reported  an  interesting  case  of  a  progressive 
muscular  atrophy  in  a  child  ;  but  they  nave  not  attempted  to  classify  it  under 
any  special  type.  The  disease  began  in  the  lower  extremities,  and  gradually 
extended  to  the  upper,  involving-  both  upper  extremities  and  the  muscles 
of  the  back.  The  gradual  changes  are  well  represented  in  Figs.  87-89. 
The  child  exhibited  some  disturbances  of  sensation  (hyperalgesia)  ;  marked 
paresis  in  neck,  back,  and  abdomen — most  marked  in  loins,  buttocks,  and 
legs  ;  least  marked  in  shoulders  and  arms  ;  no  hypertrophy  of  any  muscles. 
Electrical  reactions  at  first  little  altered  ;  later  on  faradic  excitability  consid- 
erably impaired  ;  more  markedly  in  the  legs  than  in  the  arms.  The  report 
of  the  galvanic  responses  is  too  uncertain  to  permit  of  positive  inferences  ;  the 
disease  was  steadily  progressive.  The  case  is  all  the  more  unique,  as  the 
authors  found  a  spinal  lesion  and  only  very  slight  changes  in  the  peripheral 
nerves.* 

Etiology, — Nothing  more  need  be  said  upon  this  head 
than  may  be  inferred  from  the  previous  histories.  The 
disease  is  evidently  a  family  affection,  sometimes  beginning 
at  a  very  early  age,  as  in  my  own  cases,  or  appearing  as 
late  as  the  age  of  twenty,  as  in  the  one  case  described  by 
Charcot-Marie.  Whether  the  cases  described  by  Osier  as 
occurring  in  the  Farr  family  of  Vermont,  which  set  in  as 
late  as  the  age  of  forty-six  in  some  of  the  subjects,  belong 
to  this  category  or  not  is  questionable,  as  the  cases  were 
reported  before  this  special  type  of  progressive  muscular 
atrophy  was  known.  In  both  my  cases  a  thorough  drench- 
ing of  the  skin  by  exposure  to  wet  was  mentioned,  but  I  can- 
not attribute  any  further  importance  to  this  fact  than  that 
it  may  have  helped  to  hasten  a  disease  which  was  latent  in 
the  system's  of  the  boys. 

Diagnosis. — The  diagnosis  of  this  "leg  type,"  or  neural 
form,  of  progressive  muscular  atrophy  rests  upon  the  recog- 
nition of  the  early  beginning  of  the  disease  and  of  its  hered- 
itary or  family  character.  Moreover,  the  paralysis  begin- 
ning in  the  leg  muscles,  and  spreading  to  the  upper  extrem- 

*The  case  appears  to  bear  a  close  resemblance  to  the  peroneal  form,  as  well  as  to 
an  hereditary  spinal  form. 


PROGRESSIVE.    Ml  SCULAR  ATROPHIES. 


3r>5 


/     u 


■-   o 
v    z 


366  THE  NERVOUS  DISEASES   OF   CHILDREN. 

ities  is  associated  with  slight  changes  in  sensation  in  the 

legs  as  well  as  in  the  arms.  The  occurrence  of   double 

club-foot  (not  congenital)  will  help  to  make  the  diagnosis 
still  more  certain. 

The  disease  will  have  to  be  differentiated  from  hereditary  ataxia,  from 
chronic  multiple  neuritis,  from  poliomyelitis,  and  from  the  primary  muscular 
dystrophies. 

From  hereditary  ataxia  the  disease  can  be  readily  distinguished  by  the 
absence  of  the  peculiar  unsteadiness  in  walking  and  standing,  by  normal 
electrical  reactions  in  cases  of  hereditary  ataxia,  and  by  the  persistence  of  the 
reflexes  in  many  of  the  cases  of  the  peroneal  form. 

From  chronic  multiple  neuritis  we  can  distinguish  the  "  leg  type  "  by  the 
fact  that  pain  plays  an  even  greater  role  in  most  cases  of  neuritis  ;  that  the 
atrophy  is  not  steadily  progressive,  and  that  neuritis,  of  however  long  stand- 
ing, rarely  leads  to  double  club-foot ;  and  furthermore,  that  neuritis  is  not  apt 
to  occur  as  a  family  affection. 

From  poliomyelitis  we  can  differentiate  these  cases  by  the  very  gradual 
development  of  the  disease,  in  contrast  to  the  more  sudden  onset  in  infantile 
spinal  paralysis  ;  by  the  progressive  development  of  the  atrophy,  in  contra- 
distinction to  the  retrogressive  character  of  the  wasting  in  poliomyelitis. 
Poliomyelitis  is  not  an  hereditary  or  family  disease,  and  if  the  wasting  in  a 
case  of  poliomyelitis  is  as  great  as  in  cases  of  the  leg  type  of  progressive 
muscular  atrophy  the  knee-jerks  and  other  deep  reflexes  will  surely  be  lost, 
while  in  the  leg  type  they  may  be  preserved  for  a  considerable  period  of  time. 
It  is  not  so  easy  always  to  distinguish  these  cases  from  the  subacute  forms  of 
poliomyelitis ;  but  if  there  is  any  doubt  about  the  diagnosis  in  the  earlier  stages 
of  the  disease,  the  further  progress  of  the  trouble  will  remove  all  uncertainty. 

The  disease  might  also  be  confounded,  in  its  later  stages,  with  the  Aran- 
Duchenne  type  of  progressive  muscular  atrophy,  particularly  if,  as  sometimes 
happens,  the  atrophy  in  those  cases  attacks  the  legs  very  soon  after  it  has  be- 
gun in  the  upper  extremities.  Under  such  conditions  we  must  rely  for  the 
differential  diagnosis  upon  the  fact  that  the  Aran-Duchenne  type  begins,  as 
a  rule,  much  later  in  life ;  that  it  is  rarely  of  an  hereditary  character,  and 
that  sensation  is  never  affected  in  those  cases  as  it  is  in  the  cases  now  under 
consideration.  But  I  must  concede  that  the  clinical  resemblance  may  be  so 
strong  between  these  two  forms  of  disease  that  it  will  be  practically  impossi- 
ble to  differentiate  between  them. 

Pathology. — For  a  number  of  years  after  this  disease 
was  first  described  the  morbid  anatomy  was  based  upon 
mere  speculation.  Reference  was  made  by  several  authors, 
among  them  by  Hoffmann,  to  the  older  records  of  post-mor- 
tem examination  by  Virchow,  Friedreich,  and  others.  In 
these  cases  the  authors  found  a  degeneration  of  the  nerves 


PROGRESSIVE  MUSCULAR  ATROPHIES.  367 

and  a  degeneration  of  the  columns  of  Goll,  but  no  satis- 
factory statement  could  be  made  at  that  time,  which  ante- 
dated the  discovery  of  modern  staining  methods,  regarding 
the  disease  of  the  anterior  ganglion  cells.  I  objected  to  the 
use  of  these  older  records  as  a  proof  of  the  non-involvement 
of  the  spinal  cord,  but  all  doubt  regarding  the  origin  of  at 
least  some  of  these  cases  has  been  removed  by  the  studies 
of  Marinesco,  of  Dubreuilh  {Revue  de  Mtdecine,  1890^.441) 
in  a  very  typical  case  of  a  child  dying,  in  a  family  of  which 
the  mother  and  eleven  children  were  affected  with  the  same 
trouble.  Dubreuilh  proves  that  there  were  old  changes  in 
the  peripheral  nerves,  particularly  in  the  motor  nerves  of  the 
hands  and  feet,  and  that  these  changes  diminished  toward 
the  spinal  cord.  The  gray  substance  of  the  spinal  cord 
was  normal ;  there  was  a  slight  increase  of  the  glia  in  the 
column  of  Goll,  but  not  a  true  sclerosis,  and  the  nerve-fibres 
were  not  diminished.  The  changes  in  the  muscles  consisted 
of  simple  atrophy  of  the  fibres,  of  a  loss  of  transverse  stri- 
ation,  and  of  a  proliferation  of  the  nuclei.  There  were  also 
some  degenerated  fibres  and  some  in  a  condition  of  hyper- 
trophy. It  will  be  seen  from  this  post-mortem  account  that 
the  changes  in  the  spinal  cord  were  evidently  secondary  to 
those  in  the  peripheral  nerves ;  furthermore,  that  the  changes 
in  the  muscles  resembled  more  closely  those  occurring  in 
the  primary  dystrophies  than  in  diseases  due  to  spinal  proc- 
esses. This  form  evidently  holds  a  median  position  between 
the  true  spinal  amyotrophies  and  the  primary  myopathies. 
(Cohn,  Cassirer,  and  Oppenheim  hold  this  same  view.)  The 
purely  neural  origin  of  the  disease  is  in  doubt.  Later  in- 
vestigations may  lend  some  coloring  to  the  proposition  of 
Bernhardt  to  speak  of  the  cases  as  spinal  neuritic  atrophies, 
since  the  ganglion  cells  of  the  anterior  horns,  the  anterior 
nerve-roots,  and  the  peripheral  nerves,  after  all,  constitute 
a  physiological  unit.  Further  post-mortem  examinations  of 
the  subjects  of  this  disease  may  exhibit  more  considerable 
changes  in  the  spinal  cord  than  those  which  were  found  in 
Dubreuilh's  case.  I  cannot  consider  the  question  settled, 
in  spite  of  the  autopsies  recorded  by  Siemerling,  Sainton, 
and  others,  and  would  urge  a  careful  and  detailed  examina- 
tion of  every  such  case  if  opportunity  presents  itself. 


368  THE  NERVOUS  DISEASES    OF   CHILDREN. 

The  general  treatment  of  these  cases  will  be  referred  to 
in  connection  with  the  discussion  of  the  other  forms  of  mus- 
cular disturbance.  I  only  wish  to  insist  once  more  on 
the  fact  that  the  orthopedic  surgeon  may  in  these  cases  pro- 
cure moderate  relief,  at  least  for  a  considerable  period  of 
time,  by  such  measures  as  were  employed  by  Dr.  Gibney 
in  the  cases  of  the  two  boys  under  my  immediate  care. 

PRIMARY   MYOPATHIES   (PRIMARY   MUSCULAR   DYSTROPHIES). 

Our  knowledge  of  these  dystrophies  is  a  recent  acquisi- 
tion for  which  we  are  specially  indebted  to  the  brilliant 
clinical  and  pathological  studies  of  Erb.  The  German 
neurologist  not  only  described  a  new  form  of  progressive 
myopathy,  but  demonstrated  very  clearly  the  relations  of 
this  form  to  the  older  and  well-known  form  of  muscular 
pseudo-hypertrophy.  In  addition  to  this,  Erb  has  sub- 
jected the  innumerable  types  of  muscular  atrophy  to  a 
healthful  criticism,  and  has  thus  helped  to  correct  many  er- 
roneous views  which  were  advanced  by  others.  Excellent 
work  has  also  been  done  by  Charcot,  Landouzy,  Dejerine, 
Schultze,  Striimpell,  Dercum,  Spiller,  and  others. 

For  a  long  time  the  Aran-Duchenne  type  of  progressive  muscular  atrophy 
and  muscular  pseudo-hypertrophy  were  the  only  well-known  forms  of  pro- 
gressive muscular  disease.  With  the  evidence  which  proved  that  pseudo-hy- 
pertrophy was  never  of  spinal  origin,  a  wide  distinction  was  created  between 
the  spinal  forms  and  the  primary  myopathies.  As  new  types  of  muscular 
diseases  were  described  by  individual  authors  the  question  arose  in  each  in- 
stance, whether  the  cases  reported  inclined  rather  to  the  spinal  than  to  the 
pseudo-hypertrophic  form.  It  is  only  very  recently  that  sufficient  evidence 
has  been  brought  forth  to  show  that  all  the  primary  myopathies  are  closely 
related  to  one  another,  and  that  the  types  that  have  been  so  carefully  de- 
scribed in  former  years,  are  practically  nothing  but  peculiarities  in  the  topo- 
graphical distribution  of  diseases  which  should  "be  included  under  the  broad 
term  of  progressive  muscular  dystrophies.  Much  was  at  one  time  made  of 
the  occurrence  of  atrophy  or  hypertrophy,  but  less  importance  is  attached  to 
this  point  now,  for  we  know  that  the  hypertrophic  stage  represents  in  most 
instances  an  earlier  stage  of  the  disease,  and  that  atrophy  rapidly  supervenes 
upon  this  apparent  or  real  hypertrophy  of  muscular  tissue.  Hypertrophy 
never  occurs  in  the  spinal  forms  of  muscular  wasting,*  and  in  this  respect  it 

*  Single  hypertrophied  fibres  have  been  found  in  sections  of  muscles  from  cases  of 
progressive  amyotrophy. 


PROGRESSIVE   MUSCULAR   ATROI'Jfl I-.S. 


y«j 


is  a  most  significant  symptom,  but  its  occurrence  is  not  a  sufficient  basis  for 
a  classification  of  the  various  types  of  primary  myopathy. 

Before  entering  upon  a  detailed  account  of  the  primary 
myopathies,  it  will  be  well  to  state  the  chief  features  of 
the  various  types.  By  placing  the  symptoms  in  parallel 
columns  the  entire  subject  can  be  understood  more  readily: 

Types  of  Primary  Dystrophies. 


Part  first  affect- 
ed. 

Distribution    of 
hypertrophy. 

D  i  s  t  r  i  b  u  tion 
of  atrophy. 


Parts  remain- 
ing normal. 


Muscular     Pseudo-hv- 
pertrophv. 


Legs  (calves). 


Calves,  rarely  thighs. 


Thighs,  deep  muscles 
of  back,  shoulder,  and 
scapular  muscles. 
Calves  during  later 
period  ;  at  that  time 
also  general  atrophy. 

Face,  forearm,  and 
hand,  except  in  last 
stages. 


Juvenile  form  of  Pro-         Type  Landouzy- 

GRESS1VE     MUSCULAR  DeJK 

ATROPHY      (ERB'S     TYPE).        ("INFANTILE   FORM.") 


Shoulder  girdle. 


Face   and 
girdle. 


shoulder- 


Muscles  around  shoul-    None. 

der-girdle  and  pelvic 

girdle. 
Thighs,  deep  muscles 

of  back,  upper  arm. 

Hypertrophied  parts 

may  become  atrophic 

in  later  stage. 


Face  muscles,  includ- 
ing lips  and  orbicu- 
lar i  s  palpebrarum  ; 
shoulder  and  scapu- 
lar muscles. 


Face,  forearm,  hand 
and  leg  muscles,  ex- 
cept in  last  stages. 


Forearm,  hand  and 
legs,  and  deep  mus- 
cles of  back. 


If  we  add  to  the  above  table  the  facts  that  in  all  these 
three  forms  there  is  a  distinct  history  of  heredity,  or  at  least 
of  the  occurrence  in  various  members  of  the  same  family  ; 
that  there  are  often  slight  and  sometimes  very  marked  quan- 
titative changes  in  the  electrical  reactions,  but  that  there  is 
rarely  if  ever  a  complete  reaction  of  degeneration  ;  if  we 
note,  furthermore,  that  the  reflexes  may  remain  preserved 
for  a  considerable  period  of  time  and  then  disappear,  in 
keeping  with  the  progress  of  the  muscular  wasting,  we 
shall  see  that  there  is  practically  no  other  distinction  be- 
tween these  various  types  of  myopathies  than  the  mere 
distribution  of  atrophy  or  hypertrophy.  The  hereditary 
type  of  progressive  muscular  atrophy  as  it  was  described 
by  Leyden,  is  not  included,  simply  because  heredity  is  not 
a  sufficient  basis  of  classification,  and  many  of  his  cases 
would  belong  more  properly  either  under  the  heading  of 
Erb's  type,  or  under  that  of  the  peroneal  form  of  progres- 


370 


THE  NERVOUS  DISEASES    OF   CHILDREN. 


sive  muscular  atrophy.  There  appears  at  first  sight  to  be 
a  broad  gap  between  the  cases  of  muscular  pseudo-hyper- 
trophy and  the  cases  of  Erb's  type,  but  these  different  types 
may  be  represented  in  various  members  of  a  single  family. 
The  relation  of  these  types  to  one  another  is  convincingly 
demonstrated  by  the  cases  of  three  brothers  to  be  referred 

to  in  this  chapter.     (Figs.  90- 
92.) 

Landouzy  -  Dejerine  have 
insisted  on  the  right  of  their 
type  to  special  consideration, 
claiming  that  ordinary  muscu- 
lar pseudo-hypertrophy,  and 
even  Erb's  type  of  disease, 
were  never  associated  with 
an  involvement  of  the  face, 
yet  it  is  very  certain  that  their 
type  is  practically  nothing 
more  than  that  described  by 
Erb,  plus  involvement  of  the 
face  muscles.  Landouzy-De- 
jerine  also  denied  that  an  at- 
rophy of  the  face  muscles 
was  ever  associated  with  typ- 
ical pseudo-hypertrophy  ;  but 
Westphal  first  published  a 
case  which  showed  that  the 
face  muscles  are  occasionally 
affected  in  cases  of  typical 
pseudo  -  hypertrophy,  and  I 
was  able,  some  years  ago,  to 
record  a  case  which  was  a 
typical  representative  of  mus- 
cular pseudo- hypertrophy, 
which  passed  through  the 
stage  defined  by  Erb's  type, 
and  in  which  the  muscles  of 
There  is,  therefore,  no  suffi- 
cient reason  to  retain  the  Landouzy-Dejerine  type  as  a  sep- 
arate form  of  disease. 


Fig.  90.  —  Oldest  Brother,  aged  Sixteen 
Years,  exhibiting  Atrophy  following 
Pseudo-Hypertrophy  of  the  Calves, 
and  Extreme  Atrophy  of  Shoulder 
Muscles.  Boy  unable  to  move  from 
chair  or  to  hold  himself  erect  (late 
stage  of  "pseudo-hypertrophy").  Figs. 
106-108  represent  three  brothers. 

the  face  were  also  involved 


PROGRESSIVE  MUSCULAR  ATROPHIES. 


371 


The  characteristic  symptoms  of  the  various  types  may 
now  be  described. 

Muscular  Pseudo-hypertrophy. — As  described  many 
years  ago,  above  all  by  Meryon,  Duchenne,  and  Gowers, 
pseudo-muscular  hypertrophy  is  characterized  by  its  oc- 
currence in  early  youth. 
Boys  are  affected  some- 
what more  frequently 
than  girls  ;  but  although 
affecting  boys,  the  dis- 
ease is  inherited  almost 
invariably  through  the 
mother.  The  first  symp- 
toms are,  a  weakness  in 
the  muscles  of  the  leg 
and  an  early  increase  in 
the  size  of  the  calf  mus- 
cles. In  rare  instances 
the  hypertrophy  may  be- 
gin in  the  thigh  muscles. 
The  gait  is  waddling,  and 
the  child  soon  finds  diffi- 
culty in  walking  up  and 
down  stairs,  in  climbing 
on  chairs,  in  rising  from 
the  floor  or  from  any  re- 
cumbent posture.  In  the 
earlier  stages  of  the  dis- 
ease the  patient  rises  from 
the  floor  by  dint  of  great 
effort  (see  Fig.  109)  and 
by  "  climbing  up  upon 
himself."  In  later  stages 
of  the  disease  the  patient, 
if  put  on  the  floor,  lies 
absolutely  prostrate  and 
is  not  even  able  to  raise 
the  head  from  the  floor. 

Sitting  up  without  support  may  be  entirely  impossible.    As 
the  weakness  and  atrophy  increase,  the  patient  becomes  more 


Wf~;>  v*> 

Wm 

Bfc  -  - 1 

E  ■ 

. 

i  1    m 
1 

m     ^1    *         H 

W' 

M.       ^H 

.  M^i ' 

'^w         M 

Wb  '**■ .^ttf 

ly.'ffi^Lv  L^y 

Fig.  91.  —  Second  Brother,  aged  Thirteen  and 
a  Half  Years,  exhibiting  Hypertrophy  of 
Calves,  of  Gluteal  Muscles,  and  of  Muscles 
about  the  Shoulder  Girdle  ;  Distinct  Atrophy 
of  Arm  Muscles  (Erb's  Type,  or  Juvenile 
Form  of  Progressive  Muscular  Atrophy). 
All  the  muscles  are  now  beginning  to  atro- 
phy ;  boy  is  only  a  little  less  helpless  than  his 
older  brother. 


372 


THE  NERVOUS  DISEASES    OF   CHILDREN. 


and  more  helpless,  is  unable  to  stand  or  to  walk,  becomes 
either  bedridden,  or  is  compelled  to  sit  in  a  chair  and  even 
loses  the  use  of  the  upper  extremities  ;  is  not  able  to  raise 


Fig.  92.— Youngest  of  the  Three  Brothers,  in  the  Earlier  Stage  of  Pseudo-Hypertrophy. 
(For  the  photographs  of  this  boy  I  am  indebted  to  the  courtesy  of  Dr.  Collins.) 


the  arms,  and  may  have  no  use  of  any  of  the  muscles,  except 
the  small  muscles  of  the  hand.  In  the  two  boys  (Figs.  90, 
91)  under  my  observation,  a  climbing  up  of  the  hand  along 


PROGRESSIVE  MUSCULAR   ATROPHIES. 


373 


the  head  in  order  to  get  the  arm  into  the  erect  position  was 
a  very  characteristic  feature. 

In  addition  to  the  hypertrophy  of  the  calf  muscles,  we 
now  know  that  there  is  apt  to  be  atrophy  of  the  muscles  of 
the  thigh,  of  the  arm,  and  the  shoulders  ;  the  scapular  mus- 


FiG.    93.— Boy  with   Pseudo-Hypertrophy  attempting   to  straighten  himself.     Same 

patient  as  in  Fig.  92. 

cles,  and  at  a  very  early  stage  of  the  disease  the  serrati,  the 
latissimus  dorsi,  and  the  pectoralis  major,  are  often  wasted. 
The  forearm  muscles  and  the  hand  muscles  are  rarely  af- 
fected. The  disease  is  often  associated,  as  the  other  forms 
of  myopathy  may  be,  with  symptoms  of  a  general  degener- 
ation ;  thus  I  have  found  nystagmus,  lisping  speech,  and  a 
moderate  degree  of  imbecility  in  not  a  few  of  these  cases. 


374 


THE  NERVOUS  DISEASES   OF  CHILDREN. 


Erb's  Type,  or  the  Juvenile  Form  of  Progressive 
Muscular  Atrophy. — According  to  Erb's  own  summary, 
this  type  is  characterized  by  progressive  wasting  with 
weakness  of  certain  groups  of  muscles,  beginning  either  in 
childhood  or  early  youth,  involving,  as  a  rule,  the  muscles 
of  the  shoulder  girdle,  the  upper  arm,  the  pelvic  girdle,  the 
thigh,  and  the  back  ;  the  forearm  and  leg  muscles  remain- 
ing intact  for  a  very  long  time.  The  atrophy  may  be  asso- 
ciated with  true  or  pseudo-hypertrophy  of  some  muscles. 

The  pectorals,  trapezii,  la- 
tissimi  dorsi,  the  serrati,  the 
rhomboids,  as  well  as  most 
of  the  upper-arm  muscles 
and  supinators  are  apt  to 
be  wasted  ;  while  the  del- 
toids, supraspinal,  and  in- 
fraspinati  are  either  normal 
for  a  long  time  or  hyper- 
trophied.  There  are  no 
fibrillary  contractions  and 
no  reaction  of  degenera- 
tion ;  no  sensory  or  visce- 
ral disturbances.  (See  Fig. 
91.) 

A  few  years  ago,  I  was 
inclined  to  consider  Erb's 
type  a  great  rarity  in  this 
country;  it  is  unquestion- 
ably the  least  frequent  of 
all  the  forms  of  progressive  muscular  atrophy  which  we 
have  occasion  to  see  in  clinics  or  private  practice ;  but  I 
have  seen  at  least  a  dozen  cases  of  this  form  within  a  period 
of  five  years,  from  which  the  reader  may  gather  the  fre- 
quency or  infrequency  of  the  disease;  but  there  is  no  doubt 
that  many  cases  exist  which  have  not  been  reported,  simply 
because  they  have  not  been  properly  recognized. 

The  Facio-Scapulo-Humeral,  or  Landouzy-Deje- 
rine  Type. — This  type  includes  cases  in  which  the  atrophy 
begins  early  in  life,  and,  as  a  rule,  in  the  muscles  of  the  face, 
giving  rise  to  what  the  authors  have  termed  the  "fades  my- 


Fig.  94. — Patient  with  Landouzy-Dejerine 
Type.  Indication  of  bouche  de  tapir ; 
patient  cannot  show  upper  teeth,  nor 
close  eyes.     (See  also  Fig.  8.) 


PROGRESSIVE  MUSCULAR  ATROPHIES.  375 

opathique /"  the  lips  are  considerably  thickened  and  consti- 
tute the  bouche  de  tapir,  or  tapir  mouth.  Later  on  in  the 
course  of  the  disease  the  atrophy  spreads  to  the  shoulder 
and  arm  muscles.  The  supraspinati  and  infraspinati,  the 
subscapularis,  and  flexors  of  the  hands  and  fingers  remain 
normal.  Among  these  muscles  that  remain  normal  it  may 
at  once  be  noted  are  several  which  are  distinctly  hypertro- 
phied  in  Erb's  type.  In  the  Landouzy-Dejerine  type  the 
muscles  of  deglutition,  of  mastication,  the  respiratory  and 
laryngeal  muscles,  as  well  as  the  ocular  muscles,  remain  nor- 
mal. In  exceptional  cases  the  disease  may  begin  in  the 
shoulder  or  arm  muscles,  or  even  in  the  lower  extremities. 
The  disease  is  distinctly  hereditary.  Fibrillary  contrac- 
tions and  reaction  of  degeneration  are  never  present. 

I  have  given  these  symptoms  as  nearly  as  possible  as 
they  were  stated  by  the  authors  themselves  in  order  to  do 
full  justice  to  their  cause,  but  it  will  be  evident,  without 
further  argument,  that,  with  the  exception  of  the  involve- 
ment of  the  face,  there  is  very  little  distinction  between 
these  cases  and  those  of  the  juvenile  form  of  progressive 
muscular  atrophy. 

The  various  types  of  progressive  myopathy  are  suffi- 
ciently illustrated  by  Figs.  90-94,  which  supply  additional 
evidence  in  favor  of  the  intimate  kinship  existing  between 
these  various  types. 

Figs.  90  and  91  represent  two  brothers  who  had  been  under  my  obser- 
vation for  many  years ;  the  one  is  an  example  of  muscular  pseudo -hyper- 
trophy with  atrophy  of  shoulder  and  trunk  muscles  ;  the  other  boy's  disease 
began  as  a  muscular  pseudo-hypertrophy,  but  the  hypertrophy  of  the  muscles 
above  the  shoulder  and  pelvic  girdles  constitute  it  a  most  pronounced  case  of 
Erb's  type — the  juvenile  form  of  progressive  muscular  atrophy.  A  third 
brother  was  seen  in  the  state  of  pseudo-hypertrophy  (Fig.  92).  After  a  lapse 
of  years  the  pseudo-hypertrophy  may  disappear,  and  all  three  brothers  will 
then  bear  the  closest  resemblance  to  each  other.  Fig.  94  is  a  photo- 
graph of  the  patient  afflicted  with  the  Landouzy-Dejerine  type  of  muscular 
atrophy. 

To  these  I  wish  to  add  a  brief  history  (published  in  1890),  of  a  case 
which  was  one  of  unusual  importance,  as  the  patient  presented  the  combined 
symptoms  of  all  known  types  of  primary  myopathy.  A  young  man,  twenty 
years  of  age,  whose  history  is  entirely  negative,  has  noted,  since  early  childhood, 
a  peculiarity  about  his  face ;  he  was  not  able  to  whistle  as  well  as  other  boys, 


376  THE  NERVOUS  DISEASES   OF  CHILDREN. 

and  as  long  as  he  can  remember  his  face  appeared  twisted  to  one  side,  at 
least  while  speaking.  It  was  not  until  one  year  before  my  examination  that 
he  became  aware  of  any  further  trouble.  He  claims  to  have  struck  his 
shoulder,  and  since  that  time  to  have  noticed  a  weakness  of  both  upper  ex- 
tremities. He  was  employed  by  a  surgical-instrument  maker  and  had  to  lift 
heavy  boxes,  but  this  he  is  no  longer  able  to  do. 

1  During  the  examination,  was  asked  to  whistle  ;  he  could  not  do  it,  nor 
could  he  keep  his  eyelids  tightly  pinched.  The  condition  of  his  muscles  may 
be  summarized  as  follows  : 

Wasted  :  Both  pectorals,  major  and  minor.  Both  serrati,  right  more 
than  left.  Both  latissimi  dorsi,  left  more  than  right.  Levatores  anguli 
scapulae,  right  more  than  left.  Rhomboids,  left  more  than  right.  Both  tra- 
pezii  (middle  and  lower  third),  left  more  than  right.  Both  biceps  muscles 
and  both  brachiales-antici.  Both  triceps  muscles,  right  more  than  left ;  the 
supinator  longus  of  both  arms,  right  more  than  left.  Anterior  thigh  and  leg 
muscles,  left  more  than  right.  Posterior  thigh  muscles  (thin).  Orbicularis 
palpebrarum  of  each  side  and  the  orbicularis  oris. 

Normal :  Back  muscles,  forearm  and  hand  muscles,  gluteal  muscles,  and 
muscles  of  foot. 

Hypertrophied :  Deltoids,  infraspinati,  supraspinati,  and  calf  muscles,  but 
the  right  calf  is  beginning  to.  waste. 

There  were  no  fibrillary  contractions  in  any  of  the  affected  muscles.  All 
the  muscles,  including  those  wasted  and  those  hypertrophied,  responded  to 
both  currents  in  proportion  to  the  quantity  of  normal  contractile  fibre  that 
each  muscle  retained.  There  was  no  reaction  of  degeneration  in  any  muscle. 
The  knee-jerks  were  present.  There  was  no  ataxia,  no  disturbance  of  sen- 
sation, and  there  was  not  a  single  symptom  pointing  to  an  involvement  of  the 
central  nervous  system. 

From  the  condition  of  the  calves,  there  might  be  some  reason  to  class  this 
case  among  the  pseudo-hypertrophies  ;  from  the  appearance  of  the  shoulder- 
girdle  and  the  thinness  of  the  upper  arm,  we  might  rank  it  with  Erb's  juve- 
nile form  ;  and  if  we  take  the  face  into  consideration,  we  might  classify  it 
with  the  Landouzy-Dejerine  type  of  progressive  muscular  atrophy.  It  does 
not  quite  tally  with  the  older  accounts  of  pseudo-hypertrophy ;  for  although 
the  shoulder  muscles  are  sometimes  involved  in  such  cases,  it  is  exceptional 
to  have  both  shoulder  and  face  muscles  affected.  From  Erb's  form  it  is  dis- 
tinguished by  the  involvement  of  the  face  muscles,  and  from  the  ordinary 
cases  of  the  Landouzy-Dejerine  type  this  case  is  to  be  distinguished  by  the 
additional  involvement  of  the  calves.  It  will  not  do  to  suppose  that  the  boy 
is  affected  with  three  different  diseases ;  it  is  much  more  to  the  point  to  state 
that  the  symptoms  in  this  case  prove  that  the  three  distinct  forms  practically 
represent  subdivisions  of  one  and  the  same  disease,  and  that  the  primary 
muscular  disease  was  so  fully  developed  in  this  patient  that  he  practically 
represented  all  the  known  types  of  progressive  myopathy.  We  see  by  this 
case,  too,  how  wrong  it  is  to  make  too  much  ado  over  the  varying  distribu- 
tion of  atrophies  or  hypertrophies. 


PROGRESSIVE  MUSCULAR  ATROPHIES.  3/7 

DIAGNOSIS. — It  is  much  more  difficult  to  differentiate 
between  the  various  forms  of  progressive  muscular  dys- 
trophies than  to  distinguish  between  them  and  other  dis- 
eases. The  factor  of  heredity,  the  occurrence  in  families, 
the  absence  of  fibrillary  twitchings  and  of  changes  in  elec- 
trical reaction,  and  above  all  the  slowly  progressive  char- 
acter of  these  diseases  and  their  onset  in  early  life,  will 
scarcely  permit  of  any  confusion  with  other  diseases.  If 
the  patients  are  examined  later  in  life,  after  extensive  atro- 
phy has  supervened  upon  preceding  hypertrophy,  if  the 
atrophic  paralysis  is  so  extreme  that  the  patient  is  bedrid- 
den and  that  there  are  practically  no  muscles  which  re- 
spond to  the  electrical  current,  if  all  the  reflexes  are  absent, 
there  may  be  considerable  difficulty  in  differentiating  be- 
tween such  a  condition  and  that  of  chronic  poliomyelitis. 
But  even  at  such  an  advanced  period  of  the  disease  the 
former  history  of  the  patient  will  help  to  establish  a  correct 
diagnosis. 

Mention  should,  however,  be  made  of  one  other  condition  which  I  have 
met  with  but  twice,  in  which  the  question  has  come  up  whether  the  patient 
was  suffering  from  a  form  of  primary  muscular  dystrophy.  Both  of  these 
cases  were  instances  of  what  I  would  wish  to  call  physiological  hypertrophy. 
The  one  was  the  case  of  a  physician  who  had  attained  unusual  muscular 
development  in  his  efforts  to  correct  phthisical  tendencies,  and  the  second 
was  the  case  of  a  brother  of  a  well-known  physician  who,  through  inordi- 
nate exercising  at  lawn  tennis  had  produced  a  hypertrophy  of  the  shoulder, 
arm,  and  forearm  muscles  of  the  right  side.  In  both  these  cases  I  was  con- 
sulted because  of  a  weakness  which  had  followed  upon  this  unusual  hyper- 
trophy. In  the  case  of  the  physician  first  referred  to,  several  muscles  (among 
them  the  infra  and  supra  spinati,  and  the  deltoid)  had  begun  to  atrophy  dis- 
tinctly. The  possibility  of  the  development  of  some  form  of  progressive 
muscular  atrophy  was  entertained  by  others  in  both  cases,  but  I  was  certain 
that  this  grave  prognosis  was  not  justified,  for  the  entire  development  of  the 
trouble,  the  onset  late  in  life,  and  the  occurrence  of  these  conditions  in  per- 
sons with  a  clear  family  record  furnished  the  best  evidence  that  the  condition 
of  these  two  patients  was  the  result  of  over-exercise  and  nothing  more. 

The  point  of  greatest  interest  in  both  these  cases  is  the  surprising  weak- 
ness of  the  muscles  in  spite  of  the  hypertrophied  condition.  It  would  seem 
that  any  muscular  fibre  forced  to  an  unnatural  growth  (hypertrophy)  is 
likely  to  succumb  in  the  struggle.  In  both  these  cases  I  insisted  on  absolute 
rest  of  the  affected  parts  and  on  the  use  of  common  sense  in  physical  exer- 
cise.    In  the  case  of  the  physician  all  the  muscles  have  returned  to  a  normal 


378  THE  NERVOUS  DISEASES   OF   CHILDREN. 

state  and  have  remained  so  for  fully  three  years ;  in  the  second  case  the 
normal  strength  of  the  arm  has  returned,  and  there  is  no  sign  of  an  impend- 
ing atrophy. 

The  points  of  differential  diagnosis  between  the  spinal 
forms  of  progressive  muscular  atrophy  and  the  primary 
dystrophies  have  been  insisted  upon  over  and  over  again. 
The  rule  is  that  in  spinal  cases  the  affection  is  not  hered- 
itary and  generally  begins  in  the  upper  extremity  ;  there 
are  fibrillary  contractions,  and  marked  changes  in  electri- 
cal reaction ;  while  in  the  dystrophies  heredity  is  the  most 
prominent  factor,  the  diseases  begin  early  in  life,  there  are 
no  fibrillary  contractions,  and  the  electrical  reactions  re- 
main normal  or  nearly  so  throughout  the  entire  course  of 
the  disease  until  the  stage  of  extreme  atrophy  is  reached. 

After  the  consideration  of  these  cardinal  symptoms  there  should  be  no 
real  difficulty  in  distinguishing  between  these  two  principal  forms  of  progres- 
sive muscular  wasting ;  yet  cases  appear  every  now  and  then  in  which  the 
symptoms  are  so  distributed  that  it  is  impossible  to  classify  them  by  adhering 
to  these  cardinal  symptoms.  The  most  interesting  case  in  point  is  unquestion- 
ably the  one  reported  by  Striimpell.  In  this  patient  the  disease  did  not  begin 
until  the  age  of  twenty-nine,  in  the  fingers  of  the  right  hand,  but  there  was  a 
strong  history  of  heredity,  his  mother  having  suffered  from  a  similar  disease. 
The  atrophy  spread  from  the  small  muscles  of  the  right  hand  to  the  muscles 
of  the  shoulder,  and  later  on  to  the  muscles  of  the  opposite  arm.  The  deep 
muscles  of  the  spine,  the  glutei,  and  the  thigh  muscles  remained  entirely  in- 
tact, after  a  period  of  more  than  eleven  years.  The  symptoms  just  stated 
pointed  to  a  spinal  form,  rather  than  to  a  pure  myopathy  ;  the  heredity 
was,  however,  more  in  keeping  with  the  primary  dystrophies,  and  the  entire 
absence  of  fibrillary  movements  as  well  as  of  changes  in  the  electrical  re- 
actions would  have  inclined  one  to  place  the  case  in  the  category  of  primary 
myopathies.  The  histological  character  of  the  muscular  tissue  was  also  more 
like  that  described  in  cases  of  primary  myopathies,  yet  at  the  autopsy  de- 
cided changes  were  found  in  the  spinal  cord,  in  the  anterior  nerve-roots,  and 
in  a  number  of  the  peripheral  nerves.  Thus  the  impropriety  of  classing  such 
a  case  exclusively  under  one  heading  or  the  other  was  clearly  demonstrated, 
and  I  think  Striimpell  quite  right  in  insisting  that  the  chief  value  of  his  case 
was  in  showing  that  these  cases  of  progressive  muscular  atrophy,  whatever 
form  they  take,  belong  to  the  order  of  hereditary  systemic  diseases,  and  that 
it  is  largely  a  matter  of  chance  or  else  due  to  causes  still  unknown  whether 
the  peripheral  or  the  more  central  portion  of  the  second  division  of  the  mo- 
tor tract  becomes  the  chief  seat  of  the  disease.  A  case  of  Savill's  shows 
that  the  symptoms  of  an  amyotrophy  may  coexist  with  those  of  a  myopathy 
of  the  Landouzy-Dejerine  type. 


PROGRESSIVE  MUSCULAR  ATROPHIES.  379 

Pathology.— The  designations,  "  primary  myopathy," 
or  "  primary  muscular  dystrophies,"  which  have  now  been 
universally  accepted  for  the  diseases  under  consideration,  are 
meant  to  imply  that  the  origin  of  the  disease  is  in  the  mus- 
cular system  itself,  and  that  it  is  not  due  to  changes  either 
in  the  peripheral  nerves  or  in  the  spinal  cord.     With  regard 
to  the  more  recently  described  forms  of  primary  muscular 
dystrophy,  there  has  never  been  any  doubt  as  to  the  non- 
spinal  origin,  but  a  long  and  hot  discussion  was  waged  over 
the  spinal  origin  of  muscular  pseudo-hypertrophy.    In  1888  I 
analyzed  all  the  cases  of  muscular  pseudo-hypertrophy  that 
were  accessible  at  the  time,  and  found  that  of  twenty-five 
cases  which  had  been  reported,  eight  had  to  be  excluded 
because  the  spinal  cord  was  not  examined  microscopically, 
or  because  the  examination  was  not  properly   made.     Of 
the  seventeen  remaining  cases,  the  spinal  cord  and  anterior 
nerve-roots  were  found  absolutely  normal  in  twelve,  and  in 
five  others  the  changes  that  were  found  could  not  be  held 
responsible  for  the  changes  in  the  muscles.     A  similar  con- 
clusion has  been  reached  by  other  authors,  and  at  the  pres- 
ent time  no  author  of  repute  has   ventured  to  fall  back 
upon  the  older  theory  of  the   spinal  origin   of  muscular 
pseudo-hypertrophy.    Such  slight  changes  as  were  observed 
by   some— the  diminution,   for  instance,  in  the  number  of 
ganglion  cells  or  slight  changes  in  the  contours  of  these 
cells__can  now  be  sufficiently  explained  on  the  supposition 
that  all  such  changes  are  secondary  to  the  peripheral  mus- 
cular trouble. 

The  histological  condition  of  the  atrophied  or  hypertro- 
phied  muscles  has  been  studied  with  particular  care,  for  it 
was  supposed  for  a  very  long  time  that  the  diagnosis  of  a  myo- 
pathy or  of  an  amyotrophy  could  be  based  safely  enough 
upon  histological  appearances  ;  but  we  shall  see  that  this 
hope  of  finding  some  absolute  point  of  differentiation  be- 
tween these  two  conditions  can  no  longer  be  entertained. 
In  muscles  atrophied  from  spinal  lesions,  the  following  were 
supposed  to  be  the  chief  changes :  a  loss  of  striation  of  the 
muscular  fibres  and  a  narrowing  of  the  same,  an  increase 
in  the  number  of  muscle  nuclei,  and  possibly  segmentation 
of  the  nuclei,  granular  or  fatty  degeneration  of  the  fibres, 


38O  THE   NERVOUS  DISEASES    OF   CHILDREN. 

and  occasionally  globules  of  fat  between  the  muscle  fibrils. 
Some  or  all  of  these  changes  are  present  according  to  the 
length  of  time  that  the  atrophy  has  existed,  but  all  of  these 
changes  have  also  been  found  in  spinal  forms,  as  well  as 
in  primary  dystrophies.  The  increase  in  the  nuclei  is  not 
so  great  in  the  dystrophies  as  in  the  amyotrophies.  In  the 
spinal  forms  it  was  thought  that  hypertrophied  fibres  were 
never  found,  while  they  are  extremely  common  in  the 
purely  muscular  types.  But  Mueller  and  others  have  re- 
corded exceptions  to  this  rule  and  have  proved  the  presence 
of  hypertrophied  fibres  even  in  cases  of  poliomyelitis  of 
old  standing.  A  large  number  of  hypertrophied  fibres  in 
a  given  section  of  a  muscle  is,  nevertheless,  more  frequent 
in  the  primary  muscular  dystrophies  than  in  the  spinal 
atrophies. 

The  next  question  that  arose  was  whether  it  was  possible  by  histological 
examination  to  differentiate  between  the  various  primary  myopathies.  In 
muscular  pseudo-hypertrophy  we  have,  as  a  rule,  a  narrowing  of  the  fibres 
with  changes  in  their  contour,  granular  or  fatty  degeneration  of  the  fibrils, 
and  accumulation  of  fat  globules  between  them,  and  increase,  without  marked 
proliferation,  of  the  connective  tissue.  Hypertrophied  fibres  can  be  found 
scattered  in  between  fibres  of  normal  dimensions,  or  between  those  that  have 
evidently  undergone  atrophy.  A  slight  increase  in  the  muscle  nuclei  is  often 
found,  but  unusual  increase  is  suspicious  of  a  spinal  origin.  Jacoby  thought 
that  the  disease  consisted  in  the  main  of  a  chronic  inflammation  invading 
both  the  perimysium  and  the  muscle  tissue,  and  was  inclined  to  term  the 
process  a  myositis  progressiva  hyperplasia,  but  his  views  and  his  histo- 
logical findings  have  not  been  corroborated  by  others.  Westphal,  in  a  very 
typical  case  of  pseudo-hypertrophy,  found,  on  post-mortem  examination, 
enormous  increase  of  adipose  tissue  in  which  the  muscle  fibrils  were  nearly 
of  normal  size,  increase  of  the  interstitial  connective  tissue,  no  hyper- 
trophied fibres,  and  strands  of  connective  tissue  occasionally  passing 
through  the  fatty  parts  ;  a  few  of  the  groups  of  muscle  fibres  appearing 
to  be  strangulated  by  the  strands  of  connective  tissue.  In  sections  from 
a  case  which  I  have  recently  had  occasion  to  examine,  the  fibres  were  found 
to  be  of  varying  size  and  there  was  a  distinct  proliferation  of  the  muscle 
nuclei.  Schultze,  in  a  case  which  stands  midway  between  pseudo-hyper- 
trophy and  Erb's  juvenile  form,  found,  in  addition  to  peculiar  giant-cell  for- 
mations, a  large  number  of  fat-cells  in  the  muscular  tissue,  an  increase  of  the 
connective  tissue,  and  remnants  of  hypertrophied,  normal,  and  atrophic  fibres, 
and  an  enormous  increase  of  nuclei,  which  the  author  thought  greater  than 
in  the  ordinary  cases  of  pseudo-hypertrophy.  He  also  described  the  occur- 
rence of  vacuoles  which  were  in  all  probability  not  due  to  the  hardening 


PROGRESSIVE,   MUSCULAR  ATROPHIES. 


38l 


mm 


Fig.  95. — Changes  in  Muscular  Tissue  in  a  Case 
of  Primary  Dystrophy.  (Erb. )  a,  Above  altered 
blood-vessel ;  for  other  lettering  see  text. 


process.  Hitzig's  observations  were  of  special  interest  for  a  time,  as  he 
examined  four  cases  most  carefully.  He  concluded  that  "  the  primary  and 
most  important  change  in  juvenile  atrophy  is  not  an  interstitial  process,  but 
decidedly  parenchymatous,  and  according  to  the  intensity  of  the  disease  is 
represented  by  slight  or  ex-  a 

cessive  hypertrophy  of  the 
fibres  .  .  .  The  anatom- 
ical changes  in  pseudo-hyper- 
trophy, on  the  other  hand, 
are  characterized  by  active 
changes  in  the  connective 
tissue."  This  distinction  be- 
tween the  histological  changes 
in  pseudo- hypertrophy  and 
Erb's  form  of  primary  myop- 
athy has  not  been  borne  out 
by  others,  and  if  I  am  not 
mistaken,  Hitzig  has  with- 
drawn his  former  views.  The  matter  was  definitely  settled  by  more  recent 
studies  of  Erb,  Cramer,  Pick,  and  others  (Figs.  95  and  96).  Erb  proved  that 
in  all  cases  of  progressive  muscular  dystrophies,  whether  of  one  type  or 
another,  the  changes  in  the  muscular  tissue  were  very  similar,  and  that 
such  changes  as  did  occur  were  simply  due  to  different  stages  of  the  dis- 
ease. Erb  showed  (Fig.  95)  that  hypertrophy  and  atrophy  of  fibres,  marked 
proliferation  of  the  nuclei,  vacuolization  (near  b),  and  segmentation  (c)  of 
fibres  were  the  chief  changes,  and  that  these  occurred  in  all  possible  forms  of 
primary  dystrophies,  and  of  these  phenomena  the  hypertrophy  of  the  fibres 

seemed  to  be  the  first  to  ap- 

'-^X^J^  ^^i^&§#lt&7/    pear" 

\*^*  ■■  3\-^^*^^f^n%7'./    aCJIb     '4%  s  From  a  case  of  the  Lan- 

douzy  -  Dejerine  type  (Fig. 
97),  I  excised  a  large  piece 
of  the  infra-spinatus  muscle, 
and  in  doing  this  a  piece  of 
the  nerve  as  it  enters  the 
muscle  was  accidently  re- 
moved. On  histological  ex- 
amination of  the  specimen, 
prepared  for  me  by  Dr. 
Wiener,  we  found  a  consid- 
erable amount  of  fatty  tissue  between  and  around  the  muscular  fibres  (Figs. 
97,  98)  much  atrophy,  but  no  hypertrophy  of  the  muscle  fibres;  there  was 
also  distinct  evidence  of  a  marked  degeneration  of  the  nerves.  The  finding 
was  a  very  singular  one,  but  it  will  not  do  to  draw  too  broad  an  inference 
from  a  single  case.  At  all  events,  the  participation  of  the  nerve  (whether  in 
primary  or  secondary  fashion)  in  a  disease  supposed  to  be  a  purely  muscular 
affection  is  worthy  of  note. 


FlG.  96. — a,   Normal   Fibres 


Hypertrophied 


Fibres  ;  c,  Atrophied  Fibres  and  Fat.      (Erb.) 


382 


THE'  NERVOUS  DISEASES   OF   CHILDREN. 


After  a  very  long  discussion  on  the  histological  changes 
in  progressive    muscular  dystrophies,  Erb  concludes  that 


Fig.  97. — Section  of  Infra-Spinatus  Muscle  from  a  Case  of  Landouzy-Dejerine  Type, 
showing  Deposits  of  Fat  and  Degenerated  Bundles  of  Nerves.  (Low  power.  Draw- 
ing made  after  specimen  stained  according  to  Van  Gieson.) 

these  dystrophies  are  forms  of  a  tropho- neurosis,  which 
may  occasionally  be  the  result  of  functional  disturbance  of 
trophic  centres  ;  and  that  such  disturbance  may  be  either 


\ 


<^&if. 


■*C~M 


Fig.  98. — A  Part  of  the  Nerve  Bundles  more  highly  magnified,  showing  Degenera- 
tion of  Nerve  Fibres.     (See  preceding  figure.) 

primary  or  secondary  to  spinal  lesions — a  conclusion  not 
unlike  that  reached  by  Strumpell  a  few  years'  later  in  the 
article  referred  to  above. 


PROGRESSIVE  MUSCULAR  ATROPHIES. 


3*3 


Duration. — All  these  forms  of  muscular  dystrophies  take 
a  relatively  slow  course,  but  the  period  of  time  that  elapses 
before  the  stage  of  utter  helplessness  is  reached  varies  very 
much  in  different  individuals.  It  is  safe  to  say  that  those 
suffering-  from  muscular  pseudo-hypertrophy  are  the  ones 
most  apt  to  be  completely  crippled  at  an  early  day.  Two 
or  three  years  often  suffice  to  make  the  patient  entirely  help- 
less. Erb's  juvenile  form 
and  the  Landouzy-Dejerine 
type  progress  more  slowly 
— a  number  of  years  elaps- 
ing before  the  legs  become 
involved.  I  have  seen  one 
case  of  Erb's  form  which 
has  lasted  over  thirty  years, 
and  another  of  the  typical 
muscular  pseudo-hypertro- 
phy, which  has  lasted  at 
least  fifteen  ;  but  if  death 
does  not  ensue  from  the 
disease  itself,  patients  may 
be  carried  off  by  any  slight 
intercurrent  disease. 

Treatment. —  Interest- 
ing as  these  diseases  are 
from  a  medical  and  scien- 
tific point  of  view,  they  are 
unfortunately  most  unsat- 
isfactory as  regards  the  re- 
sults of  treatment ;  and  yet 
there  is  no  need  of  despairing  and  declaring  that  nothing 
can  be  done  in  the  earlier  stages  of  these  troubles.  Con- 
tractures of  muscles  and  deformities  of  various  parts  are 
extremely  annoying  and  prevent  the  patient  from  getting 
about  freely,  or  without  the  use  of  crutches  or  wheeling- 
chairs.  I  wish  to  plead  for  the  early  and  active  interfer- 
ence of  the  orthopedic  surgeon.  As  the  diseases  are  at 
times  but  very  slowly  progressive,  the  correction  of  de- 
formities by  surgical  measures  may  bring  relief  for  a  con- 
siderable  period    of    time.     Excellent    though    temporary 


Fig.  99. — Boy  with  Defective  Development 
of  Scapula  and  Shoulder  Muscles. 


384 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


results  were  obtained  in  several  patients  whom  I  have 
treated  in  conjunction  with  Dr.  Gibney.  His  results  in  the 
cases  of  the  peroneal  form  of  progressive  muscular  atrophy 
make  one  a  little  hopeful  that  still  better  results  may  be 
attained  in  the  future  in  this  and  other  forms  of  muscular 
atrophy  by  similar  or  even  bolder  measures ;  but  as  Op- 
penheim  has  recently  insisted,  such  cases  are  not  suitable 

for  tendon  transplantation. 
Intelligent  exercise  of 
atrophied  or  hypertro- 
phied  muscles,  diligent 
and  prudent  use  of  mas- 
sage and  electricity  in  these 
cases  will  be  in  order.  The 
latter  agent  has  a  whole- 
some influence  upon  the 
condition  of  muscles  whose 
nutrition  has  been  altered, 
and  is  furthermore  a  con- 
venient form  of  moderate 
exercise.  In  the  case  of  a 
boy  suffering  from  the 
Landouzy  -  Dejerine  type, 
all  the  symptoms  were 
brought  to  a  standstill,  and 
a  decided  improvement 
has  been  effected  by  the 
careful  administration  of 
electricity.  If  the  stage  of 
atrophy  and  helplessness 
has  been  reached,  nothing  more  can  be  done  than  to  make 
the  patient  as  comfortable  as  possible. 


Fig.  ioo. 


TOTAL  ABSENCE  AND  EARLY  ATROPHY  OF  MUSCLES. 

This  condition  is  suggested  by  the  preceding  discussion 
of  the  primary  muscular  dystrophies,  and  may  as  well  be 
referred  to  in  this  connection  as  in  any  other.  The  condi- 
tion is  a  rare  one.  The  following  case,  which  I  have  seen, 
suggests  a  similar  condition,  and  at  the  same  time  shows 


PROGRESSIVE  MUSCULAR  ATROPHIES.  385 

that  the  defect  may  include  bony  parts  as  well  as  the  mus- 
cles covering  them. 

The  boy  whose  condition  is  represented  in  Figs.  99  and 
100  was  seen  by  me  a  few  years  ago.  He  was  then  six 
years  of  age.  The  mother  stated  that  she  had  noticed  dur- 
ing the  past  year  that  the  right  shoulder  was  higher  than 
the  left.  On  examination  we  found  that  there  was  shorten- 
ing  of  the  right  scapula,  the  longest  diameter  being  4  ctm. 
less  than  that  of  the  left,  and  there  was  insufficient  develop- 
ment of  the  right  supra-  and  infra-spinati  of  the  latissimus 
dorsi,  and  of  the  pectoralis  major.  It  will  be  noted  that 
these  are  the  very  muscles  which  are  often  involved  in 
cases  of  primary  muscular  dystrophies.  The  condition  is 
of  some  interest  as  showing  that  the  muscles  which  are  most 
subject  to  disease  are  also  found  congenitally  defective. 
Whether  this  defect  of  development  is  due  to  some  periph- 
eral nerve  anomaly,  or  whether  it  is  the  result  of  defective 
development  of  the  spinal-cord  cells,  cannot  be  stated. 

Dejerine  and  Thomas  have  described  a  form  of  disease 
which  they  designate  as  an  "  hypertrophic  and  progressive 
interstitial  neuritis  of  childhood."  It  bears  some  resem- 
blance to  the  peroneal  form  of  progressive  muscular  atrophy, 
and  to  hereditary  ataxia.  The  chief  symptoms  are :  Progres- 
sive wasting  of  the  muscles  at  the  distal  ends  of  the  extremi- 
ties, lightning  pains,  Romberg  symptom,  contracted  pupils, 
sluggish  reaction  of  pupils  or  complete  immobility,  nystag- 
mus, diminution  of  electrical  responses,  hardening  of  nerve 
trunks  as  determined  by  palpation.  Anatomical  findings 
were:  Chronic  interstitial  neuritis,  most  marked  at  the 
periphery  ;  sclerosis  of  the  columns  of  Goll  and  of  Burdach  ; 
atrophy  of  the  anterior  horns  and  anterior  roots. 


CHAPTER  XXII. 

MALFORMATIONS  AND  CONDITIONS  DUE  TO  DEFECTIVE 
DEVELOPMENT  OF  THE  CORD. 

Arrest  of,  or  disturbance  in,  the  development  of  the 
spinal  cord  is  generally  associated  with  similar  conditions 
affecting  the  brain.  These  spinal-cord  conditions  have,  with 
the  exception  of  spina  bifida,  very  little  clinical  interest. 
The  chief  malformations  are  the  following  : 

Amyelia,  or  Entire  Absence  of  the  Spinal  Cord,  is  associated 
with  a  condition  of  anencephaly,  and  the  absence  of  both  may  be  designated 
as  amelyencephaly.  In  some  instances  there  is  tolerably  normal  develop- 
ment of  the  cord,  however,  while  every  trace  of  the  brain  may  be  wanting.  This 
defect  of  the  spinal  cord  is  in  some  instances  a  simple  form  of  agenesis,  in 
other  cases  a  start  has  been  made  in  the  development  of  the  central  nervous 
system,  but  an  unusual  accumulation  of  cerebro-spinal  fluid  at  a  later  period 
of  foetal  life  has  destroyed  the  neural  axis.  In  forms  of  amyelia  there  is  at 
times  also  a  defective  development  of  the  vertebras.  If  the  defect  is  due  to  a 
spinal  dropsy  we  are  able  to  trace,  after  the  birth  of  the  child,  the  vertebral 
canal  and  the  spinal  membranes  which  are  distended  by  the  excessive  ac- 
cumulation of  fluid. 

•  Atelomyelia  is  the  term  by  which  the  older  authors  designated  a  par- 
tial lack  of  development,  such  as  the  entire  defect  of  the  pyramidal  tracts,  or 
a  slight  defect  in  the  substance  of  the  cord  which  occurs  at  the  site  of  the 
spina-bifida.  A  similar  defect  is  at  times  observed  in  the  lower  portions  of 
the  spinal  cord,  which  is  generally  associated  with  some  congenital  defect  in 
the  development  of  the  lower  extremities.  The  only  form  of  atelomyelia 
which  is  of  any  practical  significance,  is  the  one  referring  to  the  defective  de- 
velopment of  the  pyramidal  tracts,  and  that  has  been  considered  in  the  chap- 
ter on  hereditary  spastic  paralysis. 

Asymmetry  of  the  Cord  has  been  described  in  a  few  instances.  It  is 
probably  due  to  defective  development  of  some  of  the  systems  of  the  spinal 
cord,  particularly  of  the  pyramidal  tracts  on  one  side  of  the  cord,  and  not  on 
both. 

Heterotopia  is  a  condition  which  has  aroused  considerable  interest  of 
late.  It  denotes  malposition  of  the  gray  matter  of  the  cord  ;  parts  of  which 
are  found  scattered  among  the  systems  of  white  fibres,  either  in  the  lateral 


DEFECTIVE  DEVELOPMENT  OF   THE    CORD.  3S7 

columns  or  even  in  the  posterior  columns.  Van  Gieson  has  shown  that  het- 
erotopia can  easily  be  caused  by  careless  manipulations  of  the  spinal  cord 
during  post-mortem  removal  from  the  body.  He  throws  some  doubt  upon 
the  existence  of  any  such  condition  as  heterotopia  during  life.  While  it  may 
be  conceded  that  many  of  the  cases  that  have  been  reported  as  heterotopia, 
were,  in  all  probability,  artificially  produced,  we  cannot  deny  the  occasional 
existence  of  such  a  condition  :  for  it  has  been  found  when  the  spinal  cord  has 
been  most  carefully  removed,  and,  furthermore,  if  it  were  invariably  an  arti- 
ficial condition,  we  would  surely  find  it  in  many  more  spinal  cords  than  is 
actually  the  case. 

A  small  cord  or  micromegaly  has  often  been  noted ;  in  cases  of  hereditary 
cerebellar  ataxy  for  instance.  A  normal  adult  cord  has  a  diameter  of  6  to  9 
mm.  in  the  dorsal  vertebrae;  8  to  1 1  mm.  in  the  upper  cervical;  15  mm.  in 
the  cervical,  and  12  mm.  in  the  lumbar  enlargement. 

Diplomyelia  denotes  a  double  cord.  This  condition  is  at  times  found 
in  monstrosities  of  various  kinds,  but  occasionally  in  children  who  have  ex- 
hibited no  spinal  symptoms  during  life.  It  is  due  to  a  defective  develop- 
ment of  the  spinal  cord,  and  is  to  be  attributed  chiefly  to  an  abnormal  dilata- 
tion of  the  central  canal.  In  the  first  months  of  fcetal  life  the  central 
canal  is  relatively  wide,  and  if  normal  development  takes  place  the  canal 
becomes  smaller  and  smaller  as  time  goes  on.  If  this  retraction  does  not 
take  place,  the  substance  of  the  cord  remains  divided  and  we  practically 
have  a  double  structure.  This  division  is,  however,  never  complete,  except 
in  a  few  cases  associated  with  anencephaly.  This  diplomyelia  need' not  in- 
volve the  entire  cord.  At  times  it  is  restricted  to  a  few  segments  only.  A 
double  central  canal  is  not  a  great  rarity.  It  denotes  an  arrest  in  the  normal 
development  of  the  cord  and  as  a  rule  involves  only  a  part  of  the  medulla 
spinalis.  The  two  canals  lie  side  by  side.  The  relation  which  these  defects 
of  the  central  canal  bear  to  syringo-myelia  is  very  evident  and  need  not  be 
dilated  upon.  But  it  will  be  remembered  that  there  is  some  doubt  whether 
syringo-myelia  represents  an  arrest  in  the  development  of  the  spinal  struct- 
ures, or  a  destruction  of  parts  that  have  been  normally  developed.  If  the 
ependymal  lining  of  the  central  canal  is  entirely  normal  the  syringo-myelia  is 
likely  to  be  a  congenital  condition  and  not  one  due  to  disease.  The  disten- 
tion of  the  central  cavity  has  been  described  as  hydrorrachis  interna.  In 
contradistinction  to  this  we  have  the  condition  of 

External  Hydrorrachis  and  Spina  Bifida.  —  This 
is  by  far  the  most  important  of  all  the  congenital  anomalies 
of  the  spinal  cord,  and  is  by  no  means  a  great  rarity,  oc- 
curring in  about  one  of  every  thousand  cases.  Spina 
bifida  denotes  a  condition  in  which  there  is  an  unusual  ac- 
cumulation of  serous  fluid  in  the  vertebral  canal  either 
between  the  pia  mater  and  the  arachnoid  or  between 
the   arachnoid    and    the    dura.      Associated    with  this   in- 


388  THE  NERVOUS  DISEASES   OF   CHILDREN. 

crease  of  fluid  is  a  clett  in  the  vertebrae  due  to  an  ab- 
sence of  the  vertebral  arches,  and  in  some  instances  the 
bodies  of  the  vertebras  are  wanting.  If  there  is  no  external 
prominence  the  condition  is  called  spina  bifida  occulta. 
Recklinghausen  insists  that  the  dura  also  is  cleft  in  the  ma- 
jority of  cases.  Through  this  cleft  in  the  vertebrae  the 
spinal  membranes  or  the  cord  may  protrude,  and  accord- 
ing to  the  parts  protruded  we  can  distinguish  three  kinds  of 
spinal  hernia  :  First,  meningocele  ;  second,  meningo-myelo- 
cele  ;  third,  syringo-myelocele.  The  first  two  are  much 
more  common  than  the  last  named.  The  annexed  figures 
will  illustrate  the  condition  better  than  any  verbal  descrip- 


«  b  c< 


a  bca 


Fig.  ioi.— Meningocele,  Meningomyelocele,  and  Syringo-Myelocele. 
walls  ;  b,  cord  ;  c,  membranes.     (Dana.) 


vertebral 


tion  would.  In  all  of  the  cases  the  wall  of  the  sac  is  lined  by 
the  arachnoid,  but  not  invariably  by  the  dura.  The  pia  is 
not  part  of  the  sac-wall  unless  there  is  also  an  internal 
hydrorrhachis  or  hydromyelus.  In  some  of  them  the  spinal 
cord  as  well  as  the  nerve-roots  protrude  into  the  sac.  In 
others  again  only  a  few  nerves  are  found  in  it,  the  tumor 
contains  cerebro-spinal  fluid,  some  connective  tissue,  and,  as 
a  rule,  considerable  fat. 

Etiology. — Spina  bifida  is  caused  by  defective  closure 
of  the  vertebral  arches,  formed  from  the  mesoblast. 

This  is  a  primary  defect  in  development,  and  not  caused 
by  dropsy  of  the  spinal  cord,  as  was  once  supposed  to  be 
the  case. 

Normally  the  vertebral  column    is  closed  from  above 


DEFECTIVE  DEVELOPMENT  OF   THE    CORD.  389 

downward.  It  is  natural,  therefore,  that  the  defect  should 
be  found  most  frequently  in  that  part  (the  lumbar  seg- 
ments) which  is  the  last  to  close.  The  external  tumor  is 
generally  to  be  seen  in  the  region  of  the  second  to  fifth 
lumbar  vertebras.  There  is  distinct  fluctuation  in  the  ma- 
jority of  cases  ;  the  size  may  vary  from   that  of  an  egg  to 

the  size  of  a  child's  head.  The 
skin  of  the  tumor  may  be  normal ; 
in  some  cases  it  is  thinned  out, 
particularly  if  the  tumor  grows 
rapidly.  Ulcers  are  occasionally 
formed  ;  the  skin  may  be  ruptured, 
but  the  dura  continues  to  act  as 
the  wall  of  the  sac.  In  many  cases 
there  is  a  narrow  opening  between 
the  sac  and  the  spinal  canal.  At 
times  there  is  a  complete  constric- 
tion at  this  point,  giving  rise  to 
an  ordinary  cystic  formation  which 
may  contain  some  of  the  spinal 
structures  that  have  been  separated 
from  the  main  contents  of  the  spi- 
nal canal. 

Symptoms.  —  The  chief  symp- 
toms are  those  of  increased  press- 
ure in  the  spinal  and  cranial  cavi- 
ties, and  such  as  point  to  a  direct 
interference  with  the  functions  of 
the  spinal  cord.  Children  with 
spina  bifida  are  often  poorly  devel- 

FiG.102.-ChM  of  Seven  Years,       Qped      and     mentally     deficient.       If 

with  Spina  Bifida  and  Deform-         /         ,  „  J 

ity  of  the  Feet.  the  child  lives  long  enough  it  pre- 

sents spastic  or  flaccid  paralysis  of 
the  lower  extremities  with  anaesthesia  and  with  or  with- 
out atrophy.  Talipes  varus  is  frequent.  Remak  described 
a  form  of  club-foot  associated  with  spina  bifida,  differ- 
ing from  congenital  club-foot.  The  child  is  late  in  learn- 
ing to  walk  and  has  little  or  no  control  over  the 
sphincters  of  the  bladder  and  rectum.  Ectopia  of  the 
bladder  and  other  viscera,  defective   development  of  the 


390  THE  NERVOUS  DISEASES   OF   CHILDREN. 

lower  extremities,  are  frequent  complications  of  spina 
bifida. 

Diagnosis. — The  condition  is  easily  recognized  ;  it  is  to 
be  differentiated  from  congenital  and  other  tumors  which 
occur  often  enough  in  the  lumbo-sacral  region  and  may  be 
connected  either  with  the  substance  of  the  cord  or  with  the 
spinal  membranes. 

Fibroma,  sarcoma,  myxoma,  hygroma,  and  echinococ- 
cus  cysts  are  the  forms  of  tumor  which  occur  common- 
ly in  this  region.  It  is  of  importance  to  decide  whether 
the  sac  contains  nerve  structures  or  fluid  only.  An  explo- 
ratory puncture  with  a  needle  (attached  to  an  electric  bat- 
tery) will  help  to  clear  up  the  point. 

The  course  and  prognosis  of  spina  bifida  will  vary  much 
according  to  the  extent  of  the  hernia  ;  in  some  instances, 
the  sac  ruptures  before  or  very  soon  after  birth  ;  these 
cases  are  invariably  fatal.  The  danger  of  rupture  with 
subsequent  purulent  meningitis  is  great  in  all  cases.  If  the 
opening  of  the  sac  is  closed,  the  child  may  continue  to  live 
for  a  long  period,  but  it  is  after  all  rare  for  a  person  with 
spina  bifida  to  live  to  middle  life.  If  the  sac  contains  nerve 
structures,  the  prognosis  is  less  favorable  than  if  it  contains 
fluid  only. 

Treatment. — Much  ingenuity  has  been  exercised  by 
surgeons  in  the  attempt  to  cure  these  cases.  Compres- 
sion and  ligature  (of  the  pedunculated  tumor)  have  been 
practised  in  former  years,  but  the  results  have  been  disap- 
pointing. Ahlfeld  was  probably  the  first  to  cure  a  case  of 
this  sort  by  surgical  procedure.  Of  late  years  surgeons 
have  become  much  bolder.  Bayer  and  Hildebrand,  Schede, 
Bergmann,  and  others  have  reported  a  number  of  successful 
cases.  The  meningoceles  and  the  meningo-myeloceles  are 
considered  fit  cases  for  operation  if  the  child's  health  is 
good,  and  if  there  are  no  serious  complications  such  as 
hydrocephalus,  deformities  of  the  extremities,  ectopia  of 
the  bladder  and  the  like.  In  case  the  exact  contents  of  the 
sac  are  in  doubt,  the  X-rays  may  be  used.  A  free  incision 
of  the  sac  rs  always  to  be  preferred  to  injections  into  it  of 
iodine  and  similar  fluids. 


DISEASES   OF   THE   BRAIN. 
CHAPTER  XXIII. 

MENINGITIS   AND    ENCEPHALITIS. 

Inflammation  of  the  coverings  of  the  brain,  particu- 
larly of  the  pia  (lepto-meningitis),  is  at  times  secondary 
to  disease  of  the  brain  proper.  More  often  it  represents 
the  first  and  most  important  factor  of  a  morbid  process. 
The  anatomical  designation,  meningitis,  may  be  applied 
to  a  number  of  different  diseases.  The  symptoms  are  very 
much  the  same  in  all  these  forms  of  disease,  and  such  varia- 
tion as  occurs  depends  in  part  upon  the  intensity  of  the 
process,  and  in  part  upon  the  topographical  distribution  of 
the  disease.  It  is  natural,  therefore,  to  expect  that  the  symp- 
toms of  a  traumatic  or  of  an  idiopathic  meningitis,  which,  as 
a  rule,  involves  the  convexity  of  the  brain,  will  be  different 
in  certain  respects  from  those  of  a  tuberculous  meningitis, 
which  involves  the  base  of  the  brain;  not  forgetting  that 
the  tuberculous  process  per  se  is  responsible  for  the  severity 
of  the  symptoms,  and  the  rapid  course  which  that  disease 
runs. 

We  shall  distinguish  between — I.,  Simple  acute  menin- 
gitis; II.,  Tuberculous  meningitis;  III.,  Epidemic  cerebro- 
spinal meningitis,  and  IV.,  Meningitis  due  to  various 
causes. 

SIMPLE  ACUTE   MENINGITIS. 

This  form  of  disease  occurs  after  many  infectious  dis- 
eases (pneumonia,  ulcerative  endocarditis,  erysipelas,  etc.) ; 
after  slight  or  severe  injuries,  after  insolation,  and  in  con- 
nection with  acute  nephritis.  The  symptoms  of  acute  men- 
ingitis are  very  similar  to  those  observed  in  all  the  forms, 
except  that  they  point  to  an  involvement  of  the  convexity 


392  THE  NERVOUS  DISEASES   OF   CHILDREN. 

much  more  frequently  than  to  an  involvement  of  the  base. 
The  disease  begins,  as  a  rule,  with  a  feeling  of  malaise,  with 
vertigo,  with  nausea  and  vomiting.  These  symptoms  may 
remit  for  a  few  days  or  even  a  week,  or  they  may  progress 
continuously  until  the  headaches  become  distressing  and 
the  vertigo  so  intense  that  the  child  cannot  stand,  while  the 
nausea  and  vomiting  are  repeated  at  frequent  intervals. 
The  vomiting  has  in  most  instances  the  true  cerebral  char- 
acter (projectile  vomiting).  It  occurs  both  after  taking 
food  and  independently  of  this.  If  the  vomiting  under 
these  circumstances  is  associated  with  a  clean  tongue,  its 
cerebral  origin  becomes  all  the  more  probable.  But  too 
much  stress  should  not  be  laid  upon  this  one  point,  for  gas- 
trie  disturbance  is  so  common  in  children,  and  so  often  pre- 
cedes the  onset  of  severe  cerebral  disease  that  the  presence 
of  a  coated  tongue  need  not  suggest  the  improbability  of 
cerebral  disease.  By  degrees  the  child  becomes  listless  and 
apathetic,  it  begins  to  be  drowsy,  and  sleeps  a  great  deal, 
and  its  existence  is  taken  up  with  sleeping  and  crying.  A 
convulsive  seizure  often  occurs  during  this  stage.  The  child 
becomes  irritable,  restless,  shuns  the  light  (photophobia). 
The  temperature  varies  between  ioi°  and  1040  F.f  the  pulse 
is  rapid  at  first,  then  becomes  irregular  and  slow ;  the 
pupils  are  contracted  for  a  time,  later  on  dilated.  The 
child  grows  more  and  more  unmanageable  until  the  apathy 
deepens,  and  it  finally  passes  into  a  comatose  condition. 
Before  the  condition  of  coma  is  reached  in  the  vast  major- 
ity of  the  cases  slight  rigidity  of  the  neck  sets  in,  and  the 
upper  as  well  as  the  lower  extremities  often  exhibit  marked 
spasticity.  The  deep  reflexes  are,  as  a  rule,  increased.  The 
abdomen  is  retracted ;  the  bowels  are  constipated,  and  if 
the  child  is  in  a  deeply  comatose  condition  all  efforts  at 
feeding  it,  or  at  making  it  take  the  breast,  are  entirely  un- 
availing. 

According  to  the  severity  of  the  disease  these  symptoms 
will  be  developed  in  a  shorter  or  longer  period  of  time,  but 
as  a  rule  a  week  from  the  onset  is  quite  sufficient  for  a  full 
display  of  the  symptoms.  To  the  symptoms  noted  above 
we  may  add  the  loss  of  vesical  and  rectal  control,  retention 
of  urine,  or  involuntary  micturition  and  defecation.     Vaso- 


MENINGITIS.  393 

motor  disturbances  are  marked;  taches  cerebrales  occur  in 
the  disease,  but  have  no  diagnostic  significance.  In  some 
cases  ocular  palsies  (strabismus,  ptosis)  may  be  present,  and 
the  optic  neuritis  may  give  rise  to  temporary  or  permanent 
blindness.  A  monoplegic  or  hemiplegic  paralysis  having  all 
the  symptoms  of  a  cerebral  palsy  may  be  developed.  If  the 
disease  takes  an  unfavorable  turn  all  the  symptoms  increase 
in  severity,  the  rigidity  of  the  neck  becomes  extreme ;  opis- 
thotonos is  developed;  the  blindness  continues;  the  coma 
is  deepened  ;  respiration  becomes  irregular,  the  Cheyne- 
Stokes  type  becoming  more  marked  until  respiration  ceases 
altogether.  If  the  patient  is  to  pass  on  to  recovery,  the 
symptoms  become  stationary  for  a  time  after  the  comatose 
condition  has  been  reached  ;  then  the  rigidities  are  lessened, 
the  optic  neuritis  may  recede,  and  from  day  to  day  there  are 
evidences  of  returning  consciousness. 

Etiology. — The  occurrence  of  acute  meningitis  in  as- 
sociation with  acute  infectious  diseases  will  be  referred  to 
again.  "  Idiopathic  meningitis  "  has  been  held  by  many  to 
be  a  cover  for  our  ignorance.  There  is  no  doubt,  however, 
that  meningitis  may  be  developed  without  assignable  cause 
in  a  child  that  comes  of  healthy  parents,  and  that  has  itself 
enjoyed  perfect  health  up  to  the  time  of  the  onset  of  the 
disease.  One  variety  may  be  attributed  to  the  effect  of  in- 
tense heat  (sunstroke).  It  is  quite  likely  that  a  considerable 
number  of  the  so-called  idiopathic  forms  are  due  to  injuries, 
the  traumatic  factor  being  so  slight  at  times  that  it  does  not 
receive  the  attention  which  it  merits. 

About  ten  years  ago  a  child  was  seen  in  my  clinic  that  had  fallen  from  its 
crib,  a  fact  which  the  mother  mentioned  quite  casually.  When  the  child  was 
first  examined  it  presented  the  typical  symptoms  of  the  first  stage  of  a  gen- 
eral convexity  meningitis.  It  was  slightly  stuporous,  unable  to  stand  or  sit 
alone,  the  head  was  stiff,  and  it  had  had  occasional  spells  of  vomiting.  This 
stuporous  condition  soon  passed  into  one  of  deep  coma,  the  child  lost  ground 
rapidly,  never  exhibiting,  however,  any  cranial-nerve  symptoms.  After  a 
period  of  at  least  six  weeks,  during  which  time  the  child  was  more  or  less  som- 
nolent, a  favorable  change  set  in,  consciousness  began  to  return,  and  the  child 
recovered  fully  from  what  was  evidently  a  simple  traumatic  meningitis. 

Morbid  Anatomy  and  Pathology.  —  Simple  acute 
meningitis  is  characterized  by  an  inflammatory  condition 


394  THE  NERVOUS  DISEASES   OF   CHILDREN. 

of  the  pia  mater,  which  is,  as  a  rule,  attended  by  slight  in- 
flammation  in  the  dura  and  in  the  gray  matter  of  the  brain. 
In  contradistinction  to  other  forms  this  inflammation  is  of 
a  serous,  or  at  least  of  a  non-purulent  character.  There  is 
an  increase  of  cerebro-spinal  fluid,  the  arachnoid  may  ap- 
pear a  trifle  opaque,  while  the  substance  of  the  brain  is  dis- 
tinctly cedematous,  and  even  watery.  The  ventricles  are 
distended,  and  there  may  be  a  condition  corresponding  to 
an  acute  hydrocephalus.  The  pia  of  the  convexity  of  both 
hemispheres  is  the  part  most  extensively  diseased,  the  pia 
of  the  base  often  being  entirely  free  from  all  disease,  though 
a  slightly  increased  exudation  of  lymph  may  be  apparent 
in  the  interpeduncular  space. 

On  microscopical  examination  the  blood-vessels  of  the 
pia  are  generally  found  to  be  slightly  engorged,  and  an  ex- 
travasation of  white  blood-corpuscles  is  found  in  the  vicin- 
ity of  the  blood-vessels.  If  the  disease  has  lasted  for  any 
considerable  length  of  time,  the  pia  and  the  cortical  sub- 
stances have  become  agglutinated  so  that  the  pia  cannot 
be  removed  without  tearing  the  outer  layer  of  gray  matter. 
-  Diagnosis. — The  diagnosis  of  simple  acute  meningitis 
rests  upon  the  recognition  of  the  symptoms  common  to  all 
forms  of  meningitis.  These  are  headache,  vomiting,  coma, 
and  convulsions ;  irregularity  of  the  pulse  and  unequal  pupils. 
It  is  well  to  note  also  the  absence  of  those  factors  which 
accompany  the  graver  forms  of  meningitis  (high  fever,  ba- 
silar symptoms,  rapid  emaciation,  and  a  rapid  increase  in 
all  the  symptoms).  The  differential  diagnosis  will  not  be 
an  easy  one,  and  sometimes  a  positive  diagnosis  cannot  be 
reached  until  the  disease  takes  an  unexpectedly  favorable 
turn,  or  until  the  patient  is  seen  upon  the  post-mortem 
table.  But  the  mistake  that  is  most  frequently  made  is  that 
the  meningeal  symptoms  accompanying  many  acute  infec- 
tious diseases  are  at  once  pronounced  to  be  the  symptoms 
of  true  meningitis.  This  diagnosis  has,  to  the  author's 
knowledge,  been  made  in  the  first  stages  of  typhoid  fever, 
and  in  measles  and  scarlet  fever  beginning  with  convulsions  ; 
the  error  is  common  in  otitis  and  in  infantile  spinal  paraly- 
sis that  is  attended  by  convulsive  seizures  and  high  fever. 
It  is  well  to  remember  that  though  these  symptoms  resem* 


MENINGITIS.  395 

ble  those  due  to  meningitis,  they  are  frequently  associated 
with  other  diseases,  and  that  no  physician  should  be  in  a 
hurry  to  diagnosticate  meningitis,  pure  and  simple,  unless 
other  diseases  can  be  safely  excluded. 

Course  and  Prognosis. — The  course  of  a  simple  menin- 
gitis will  almost  invariably  cover  a  period  varying  from  four 
to  twelve  weeks,  or  even  longer.  In  those  cases  that  do  not 
tend  to  recovery  the  symptoms  gradually  become  intensi- 
fied, respiration  becomes  more  and  more  difficult,  and  after 
dragging  along  for  some  weeks,  now  and  again  yielding  a 
hope  of  recovery,  the  child  finally  succumbs  from  mere  ex- 
haustion, from  some  intercurrent  disease,  such  as  bronchitis 
or  pneumonia,  or  from  the  effects  of  cystitis,  bedsores,  and 
the  like. 

In  those  cases  that  recover  after  the  coma  has  lasted  for 
a  period  varying  from  one  to  three  or  four  weeks,  signs  of 
returning  consciousness  are  noticed,  the  child  opening  its 
eyes,  voluntarily  looking  around,  again  taking  hold  of  the 
bedclothes,  of  the  hand  of  the  nurse  or  mother,  and  so  on. 
By  degrees  the  sight  improves,  hearing,  if  diminished  or  in- 
creased, becomes  normal,  the  child  begins  to  take  its  food 
properly,  and  from  week  to  week  a  distinct  improvement  is 
noticeable,  until  all  the  symptoms  have  disappeared.  But 
not  all  of  the  cases  that  recover  end  thus  fortunately.  The 
disease  does  not  necessarily  kill,  but  it  often  leaves  distinct 
traces  behind.  Not  a  few  of  those  who  are  permanently 
blind  owe  their  misfortune  to  a  meningitis  in  early  years. 
Permanent  paralysis  and  contractures  of  one  or  more  ex- 
tremities are  attributable  to  the  same  cause,  and  defective 
speech,  and,  above  all,  a  defective  intellect  are  very  often  the 
unfortunate  outcome  of  meningitis  in  early  life.  Such  de- 
formities and  defects  do  not,  however,  result  as  frequently 
from  simple  meningitis  as  from  the  severer  types,  from 
which  recovery  is,  on  the  whole,  much  rarer. 

Treatment. — In  every  form  of  acute  meningitis,  what- 
ever its  origin  may  be,  I  am  in  favor  of  adopting  the  fol- 
lowing procedure  : 

First,  keep  the  patient  absolutely  quiet,  and  in  a  semi- 
darkened  room,  and  secure  the  services  of  a  careful  and  in- 
telligent  nurse.     Give   the    child   an    efficient    purgative ; 


396  THE  NERVOUS  DISEASES   OF  CHILDREN. 

none  will  do  better  than  calomel,  which  has  attained  a  cer- 
tain dignity  in  all  diseases  of  the  central  nervous  system. 
Place  an  ice-bag  over  the  convexity  of  the  skull,  or  on  the 
nape  of  the  neck  if  the  symptoms  point  to  the  involvement 
of  the  basilar  portion.  Though  we  cannot  claim  any  direct 
therapeutic  effect  it  will  do  no  harm,  for  it  will  at  least 
help  a  little  to  reduce  the  general  rise  of  temperature,  and 
is  useful  as  the  first  point  of  attack  in  treatment.  Place  the 
child  at  once,  according  to  its  age,  upon  moderate  doses  of 
the  bromide  and  iodide  of  sodium  (three  to  five  grains  of 
each  every  four  hours).  Give  these  in  simple  water,  or 
in  milk.  This  treatment  may  well  be  persisted  in  for  a  few 
days.  If  no  effect  is  observed,  while  it  is  well  to  continue 
the  salts  of  sodium,  other  more  energetic  measures  may  be 
employed  in  addition.  Among  these  I  would  place,  first,  al- 
ternate lukewarm  and  cold  douching  of  the  nape  of  the  neck 
and  the  upper  portion  of  the  spine,  and  inunctions  of  some 
form  of  mercury,  either  of  the  ten  per  cent,  oleate  of  mer- 
cury or  of  the  unguentum  hydrargyri.  Inunctions  should 
be  performed  by  the  nurse  or  the  mother,  and  done  so 
thoroughly  that  the  mercury  disappears  into  the  skin  of 
the  patient. 

For  purposes  of  diagnosis,  as  well  as  for  therapeutic 
reasons,  lumbar  puncture  may  be  attempted  ;  in  those  cases 
in  which  the  symptoms  point  to  greatly  increased  intra- 
cranial pressure,  it  will  be  best  to  tap  the  spinal  canal  on 
successive  or  on  alternate  days,  withdrawing  only  about 
10  c.c.  at  any  one  time. 

After  the  patient  has  made  a  fair  recovery,  the  general 
condition  of  the  system  will  need  looking  after,  and  a  thor- 
ough course  of  cod-liver  oil,  of  malt,  or  of  iron,  will  be  quite 
in  order,  and  for  some  months  after  recovery  from  this 
severe  disease,  the  child  should  be  kept  free  from  all  ex- 
citement and  from  all  mental  and  physical  fatigue.  A 
restful  out-of-door  life  is  the  very  best  conclusion  to  this 
method  of  treatment.  To  patients  who  exhibit  the  least 
tendency  to  paralysis  or  contracture,  massage  should  be 
given  as  soon  as  the  acute  symptoms  have  disappeared,  and 
electric  treatment  should  be  applied  to  the  parts  that  are 
distinctly  paralyzed. 


MENINGITIS.  397 


TUBERCULOUS    MENINGITIS 

Tuberculous  meningitis  is  by  far  the  most  frequent  form 
of  meningeal  disease  in  children,  if  we  except  the  ravages 
in  recent  years,  and  in  this  community,  of  the  epidemic 
form  of  cerebro-spinal  meningitis. 

The  disease  itself  was  first  recognized  by  Robert  Whytt,  in  1768,  and 
since  that  time  has  been  the  subject  of  innumerable  articles ;  but  every  point 
regarding  the  disease  is  now  so  thoroughly  known  that  we  can  sum  up  its 
chief  characteristics  without  referring  to  individual  authors.  Since  Koch's 
discovery  of  the  bacillus  of  tuberculosis  there  has  been  no  doubt  of  the 
microbic  origin  of  tuberculous  meningitis.  According  to  Holt's  statistics, 
twenty-five  per  cent  of  all  deaths  from  tuberculosis  in  children  were  due  to 
meningitis.     This  form  of  meningitis  is  always  secondary. 

The  disease  occurs  in  children  much  more  frequently 
than  in  adults;  while  it  is  rare  in  young  infants,  it  is  more 
frequent  in  children  between  the  ages  of  six  months  and 
four  years.  In  families  in  which  there  is  a  distinct  heredi- 
tary taint  of  tuberculosis  or  scrofula,  children  that  have 
been  apparently  well  are  suddenly  attacked  by  this  malig- 
nant disease,  and  healthy  children  in  families  in  which  every 
hereditary  predisposition  is  wanting,  or,  at  least,  denied, 
are  also  affected.  But  in  the  majority  of  instances  the  chil- 
dren attacked  have  been  weak  and  feeble ;  many  of  them 
have  suffered  from  chronic  intestinal  troubles,  from  swell- 
ings of  the  glands,  from  nasal  and  aural  catarrhs,  in  short, 
from  those  conditions  from  which  we  may  safely  argue  a 
tubercular  infection.  A  chronic  laryngitis  or  bronchitis,  or 
a  swelling  of  the  bronchial  glands,  which  may  not  have 
given  rise  to  any  special  symptoms,  is  often  the  precursor 
of  tuberculous  meningitis. 

Onset. — In  contradistinction  to  other  forms  of  meningi- 
tis, that  dependent  upon  tubercular  infection  comes  on  in  a 
very  insidious  fashion.  The  child  first  loses  its  brightness 
and  cheerfulness,  and  complains  of  an  occasional  headache, 
and  slight  nausea;  a  vomiting  spell  may  occur.  The  pulse 
is  generally  rapid.  These  complaints  almost  invariably  lead 
to  the  suspicion  of  slight  gastric  disturbance,  which,  fortu- 
nately, is  quite  often  the  case  ;    but  every  physician  will 


398  THE  NERVOUS  DISEASES   OF  CHILDREN. 

do  well,  whenever  this  series  of  phenomena  occurs,  to  be 
watchful,  and  to  give  notice  that  he  should  be  informed  if 
the  symptoms  do  not  promptly  disappear.  There  may  be 
a  marked  remission  or  great  improvement  for  a  few  days  5 
then  a  change  takes  place,  the  nausea  becomes  more  frequent, 
the  headache  more  intense,  and  the  vomiting  spells  are  a 
daily  occurrence.  In  this  way  a  week  or  more  may  pass,  the 
physician  and  parents  hoping  that  all  the  symptoms  may 
subside.  At  the  end  of  about  a  week  little  doubt  remains  of 
the  significance  of  the  condition,  for  if  a  child  is  afflicted 
with  a  tuberculous  meningitis  the  headaches  become  intense 
and  persistent,  the  child  gives  the  short  hydrocephalic  cry, 
and  by  degrees  becomes  somnolent ;  slight  rigidity  of  the 
neck  is  observed,  the  pulse  has  become  slower,  and  the  child 
shows  every  sign  of  serious  illness.  Aphasia  may  be  an  early 
symptom  ;  and  all  signs  point  to  an  intense  general  brain  dis- 
turbance, and  to  the  localization  of  the  disease  chiefly  at  the 
base  of  the  brain,  from  which  the  various  cranial  nerves  issue. 
The  temperature  is  subject  to  great  variations,  and  during 
the  first  few  weeks  it  does  not,  as  a  rule,  rise  above  1030  F., 
but  during  the  last  week  it  may  reach  105°  F.,  and  during 
the  terminal  stages  may  reach  1060  F.,  or  even  higher.  In 
one  case,  an  hour  before  death,  I  recorded  a  temperature  of 
1070  F.  This  is  supposed  to  be  due  to  a  paralysis  of  the 
heat-regulating  centres.  Respiration  is  not  seriously  inter- 
fered with,  as  a  rule,  until  the  child  enters  upon  the  ter- 
minal period,  during  which  stage  the  breathing  becomes 
irregular,  often  of  the  typical  Cheynes  -  Stokes  type,  and 
cyanosis  is  added  to  the  host  of  other  symptoms. 

As  we  examine  the  child  from  head  to  foot,  we  are 
apt  to  find  a  variety  of  symptoms.  Rigidity  of  the  neck, 
with  or  without  opisthotonos,  and  excessive  painfulness  on 
every  passive  movement  of  the  head  or  trunk.  The  major- 
ity of  the  patients  present  distinct  convergent  strabismus, 
due,  as  a  rule,  to  a  paralysis  of  one  or  more  of  the  ocular 
muscles.  The  pupils  are  unequal  and  dilated,  contracting 
very  sluggishly  to  light,  and  their  reaction  during  accommo- 
dation can,  of  course,  not  be  tested  in  consequence  of  the 
comatose  condition.  The  conjunctival  reflex  is  lost  at  an 
early  period,  and  in  consequence  of  diminished  movement 


MENINGITIS.  399 

of  the  eyelids  the  cornea  becomes  cloudy  easily.  Degluti- 
tion can  be  carried  on  only  imperfectly,  and  during  an  ef- 
fort to  open  the  mouth  or  to  perform  chewing  motions,  a 
trismus  is  very  apt  to  set  in.  A  further  examination  of  the 
head  may  reveal  a  paralysis  of  some  of  the  branches  of  the 
facial,  the  paresis  of  these  muscles  being,  at  times,  unilat- 
eral and  at  other  times  bilateral ;  but  the  paresis  is  evi- 
dently due  to  involvement  of  the  nerves  at  the  base,  and  if 
an  examination  is  made  to  bring  out  this  special  point,  the 
electrical  reactions  may  be  found  altered  in  keeping  with 
this  special  localization  of  the  lesion. 

An  examination  of  the  eyes  will  disclose  a  hyperaemic 
and  swollen  condition  of  the  papillae.  In  some  instances 
there  may  be  a  typical  optic  neuritis,  single  or  double.* 

The  abdomen  is  retracted,  taches  cerebrales  are  easily 
produced  (not  pathognomonic). 

The  upper  and  lower  extremities  may  be  paralyzed  to 
a  greater  or  lesser  degree ;  the  exact  amount  of  paralysis 
cannot  always  be  easily  determined  on  account  of  the  en- 
tire absence  of  voluntary  action,  and  because  the  physician 
is  unable  to  make  satisfactory  tests  for  this  special  point. 
While  the  paralysis  may  be  of  sufficient  interest  as  illus- 
trating the  exact  distribution  of  the  meningeal  process  it  is 
of  very  little  practical  importance,  and  loses  in  value  as 
compared  with  the  other  and  more  serious  symptoms.  The 
paralysis  may  be  hemiplegic;  in  some  forms  it  is  bilateral 
or  irregular.     The  cutaneous  reflexes  are  diminished,  often 


*Much  has  been  made  of  the  presence  of  tubercles  in  the  choroid,  and  it  has  been 
claimed  by  many  that  the  existence  of  these  tubercles  is  one  of  the  most  important 
symptoms  of  the  earlier  stages  of  the  disease.  The  truth  of  this  cannot  be  gainsaid, 
but  as  a  matter  of  practical  experience  it  must  be  admitted  that  in  many  cases  of  un- 
doubted tubercular  meningitis  the  expert  oculist  does  not  find  tubercles,  and  that  in 
those  very  cases  in  which  the  discovery  of  such  a  tubercle  would  have  helped  to  de- 
termine the  exact  character  of  a  meningitis,  the  tubercles  cannot  be  seen,  although 
later  post-mortem  examination  leaves  no  doubt  of  the  tubercular  character  of  the 
process.  While  we  may  regard  the  presence  of  tubercles,  therefore,  as  a  valuable 
corroborative  symptom,  a  failure  to  detect  them  should  not  be  allowed  to  disprove  the 
diagnosis  if  other  symptoms  would  seem  to  point  to  the  tubercular  nature  of  the 
trouble.  Dr.  R.  L.  Randolph  has  examined  thirty-five  cases  of  meningitis  of  all  kinds 
with  the  ophthalmoscope,  and  found  the  fundus  normal  in  seven  patients.  The  optic 
disks  were  usually  congested,  with  the  retinal  vessels  distended  and  remarkably  tortu- 
ous In  three  cases  the  entire  eye  was  normal,  and  these  three  patients  recovered.  In 
the  four  fatal  cases  with  normal  fundus  some  other  ocular  symptoms  were  present. 


400  THE  NERVOUS  DISEASES    OF   CHILDREN. 

lost.  The  deep  reflexes  in  the  upper  and  lower  extremities  are, 
as  a  rule,  increased.    Kernig's  symptom  is  generally  present. 

Epileptiform  convulsions  are  not  rare,  both  at  the  onset 
of  the  disease  and  during  later  stages.  These  convulsions 
are,  as  a  rule,  general  in  distribution,  and  not  of  the  Jack- 
sonian  type.  If  the  latter  form  should  prevail,  there  would 
be  good  reason  to  infer  that  the  cortex  is  diseased  as  well 
as  the  base  of  the  brain.  Considering  the  fact  of  the  ex- 
istence of  so-called  epileptiform  centres  in  the  pons  and 
medulla,  the  wonder  is  not  that  such  convulsions  occur,  but 
that  they  do  not  occur  much  more  frequently.  In  the 
terminal  stages  of  the  disease  convulsions  become  more 
frequent,  as  they  do  in  many  other  brain  diseases;  and  in 
tubercular  meningitis  the  frequent  occurrence  of  convul- 
sions, with  a  rise  in  temperature,  may  be  taken  to  be  the  sign 
of  the  approaching  end.  In  the  terminal  period,  too,  the  paral- 
ysis becomes  complete,  the  pupils  are  dilated,  the  tongue  dry 
and  furred  and  the  temperature  may  fall  to  930  or  940  F., 
until  an  ante-mortem  elevation  of  temperature  begins. 

All'  the  phenomena  are  remarkably  persistent  during  the 
course  of  this  disease.  There  are  at  times  slight  remissions 
in  the  ocular  palsies,  but  after  the  symptoms  have  once 
been  fully  developed,  they  remain  very  much  the  same  to 
the  end.  The  changes  which  take  place  are  these  :  Respira- 
tion becomes  irregular,  at  times  intermittent,  the  pulse 
grows  feebler  and  slower,  deglutition  more  and  more  diffi- 
cult, and  the  child  dies  from  paralysis  of  the  cardiac  and 
respiratory  centres. 

Morbid  Anatomy  and  Pathology. — Every  one  who 
has  had  an  opportunity  to  remove  the  brain  from  cases  of 
tuberculous  meningitis  is  surprised  by  the  few  changes 
found  in  this  organ.  In  the  great  majority  of  autopsies,  on 
the  removal  of  the  calvarium  there  is  very  little  evidence 
of  any  active  process  on  the  convexity.  In  some  brains  the 
pial  vessels  are  much  congested,  the  sinuses  are  filled  with 
clots  that  have  evidently  been  formed  only  a  few  days  be- 
fore death,  and  the  hemispheres  in  general  present  a  more 
or  less  cedematous  appearance. 

Minute  tubercles  are  noticed  along  the  distribution  of 
some  of  the  larger  pial  veins  on  the  convexity.     The  chief 


MENINGITIS.  40 1 

changes  cannot  be  noted  until  the  brain  has  been  remov<  'I 
from  the  skull.  At  the  base  of  the  brain  the  character  of 
the  disease  is  easily  recognized,  the  pia  is  cloudy,  and  in 
some  places  bulging  because  of  the  accumulation  of  fluid 
underneath.  This  is  particularly  noticeable  in  the  interpe- 
duncular spaces,  in  which  the  tubercles  are,  as  a  rule,  most 
freely  developed.  These  tubercles,  often  no  larger  than  a 
pin's  head,  may  be  scattered  throughout  the  entire  pia  from 
the  optic  chiasm  to  the  pons,  medulla,  and  spinal  cord. 
The  presence  of  tubercles  is  not  always  established  at  the 
time  of  the  autopsy,  but  this  need  not  militate  against  the 
proper  diagnosis,  for  it  is  a  fact,  well  proven,  that  in  un- 
doubted tubercular  diseases  we  may  have  an  involvement 
of  the  pia  without  tubercles,  and  occasionally  the  presence 
of  tubercles  with  but  few  signs  of  an  inflammatory  process. 

Pathology. — Of  the  pathology  of  tubercular  meningitis 
little  need  be  said,  as  it  belongs  to  the  order  of  infectious 
disorders,  and  the  disease  is,  in  almost  every  instance,  a  part 
of  a  general  tubercular  infection.  The  tubercular  form  is, 
moreover,  rarely  a  primary  affection,  although  it  sometimes 
occurs  in  children  who  have  been  apparently  healthy.  A 
thorough  post-mortem  examination  reveals  quite  regularly 
distinct  foci  of  disease  in  the  lungs,  in  the  mesenteric  glands 
from  which  the  infection  in  all  probability  took  its  start.  The 
invariably  fatal  issue  must  be  attributed  in  part  to  the  gen- 
eral effect  of  the  tubercular  poison  and  its  ravages  in  other 
organs;  for  the  remarkably  slight  changes  in  some  of  the 
cases  that  have  taken  a  rapid  course  are  the  surprise  of 
every  pathologist.  A  few  small  tubercles,  without  much 
exudation,  even  though  they  be  in  the  vicinity  of  the  pons 
and  medulla,  can  hardly  be  considered  a  sufficient  cause  of 
death,  whereas  the  toxines  circulating  in  the  blood  may  have 
been  the  initial  cause  of  the  paralysis  of  the  vital  centres. 

DIAGNOSIS. — The  difficulties  of  a  differential  diagnosis* 
are  not  limited  merely  to  the  period  of  onset,  during  which 

*  The  general  practitioner  is  apt  to  attach  too  much  importance  to  the  general 
symptoms,  and  too  little  to  the  local  (basilar)  symptoms.  The  pulse,  the  tempera- 
ture, the  condition  of  the  abdomen,  may  leave  the  diagnosis  in  doubt,  but  an  acute 
ocular  palsy,  however  slight,  or  an  incipient  optic  neuritis,  will  indicate  the  true  nature 
of  the  trouble. 


402  THE  NERVOUS  DISEASES   OF  CHILDREN. 

time  the  most  experienced  physician  may  well  be  in  doubt 
as  to  the  true  character  of  the  disease.  But  the  more  diffi- 
cult question  arises  later  on,  whether  the  disease  be  a  simple 
meningitis,  whether  it  be  of  tubercular  origin,  or  whether 
it  represents  an  epidemic  form.  In  order  to  determine  this, 
it  is  best  to  keep  in  mind  the  antecedent  history  of  the 
child ;  a  history  of  tubercular  trouble  in  the  family,  or  of 
an  early  scrofulous  or  chronic  catarrhal  condition  would 
naturally  prejudice  the  physician  in  favor  of  a  diagnosis  of 
tubercular  trouble,  but  everyone  has  experienced  curious 
surprises  in  this  respect,  for  children  of  tubercular  ances- 
try may  have  a  simple  meningitis  which  is  recoverable,  or 
they  may  be  afflicted  with  the  epidemic  form.  The  diag- 
nosis will  be  confirmed  by  the  presence  of  tubercle  bacilli 
in  the  spinal  fluid,  or  according  to  French  authors,  by  the 
presence  of  mononuclear  lymphoid  cells.  The  absence  of 
bacilli  need  not  militate  against  the  diagnosis.  In  addition, 
it  is  fairly  safe  to  infer  that  cases  of  tubercular  meningitis 
invariably  present  basilar  symptoms,  whereas  the  symptoms 
pointing  to  involvement  of  the  convexity  only,  occur  more 
frequently  in  the  other  forms. 

Course  and  Prognosis. — Tuberculois  meningitis  runs, 
as  a  rule,  a  course  varying  between  three  and  six  weeks, 
though  the  time  may  be  extended  a  little  if  the  premonitory 
period  is  taken  into  account.  In  the  more  virulent  forms 
death  may  occur  at  the  end  of  the  first  week,  or  in  the 
course  of  ten  or  twelve  days ;  and  in  these  forms  I  have 
often  found  only  slight  post-mortem  changes,  from  which 
we  may  infer  that  the  general  toxine  poisoning  has  been  of 
much  more  consequence  than  the  local  deposit  at  the  base 
of  the  brain.  As  for  the  prognosis,  no  one  need  hesitate  to 
say  that  it  is  absolutely  bad;  but  in  making  such  a  prognosis 
the  most  experienced  physician  will  do  well  to  remember 
that  this  diagnosis  is  never  quite  as  certain  as  is  the  prog- 
nosis based  upon  it.  There  is  grave  doubt  whether  cases 
of  tuberculous  meningitis  ever  recover. 

Henoch,  and  Rilliet  and  Barthez,  record  two  cases  in  which  death  ensued 
from  a  second  attack,  occurring  some  years  after  the  first ;  but  even  here  there 
is  room  for  doubt  as  to  whether  the  first  attack  of  meningitis  was  truly  tuber- 


MENINGITIS.  403 

cular  in  character.  Pollitzer  reports  a  case  of  a  child  which  survived  three 
years  after  an  attack  of  basilar  meningitis  ;  at  the  autopsy  he  found,  at  the 
base,  the  distinct  evidences  of  an  old  exudation  over  the  pons,  which  was  in 
all  probability  of  a  tuberculous  nature.  Freyhan  found  the  tubercle  bacilli  in 
the  cerebro-spinal  fluid  of  a  patient  who  recovered. 

Oppenheim  has  reported  six  cases  of  a  localized  tuberculous  meningo-en- 
cephalitis  of  the  Rolandic  areas  in  children,  simulating  brain  tumor,  ending  in 
recovery.  The  anatomical  proof  is  wanting,  but  the  author's  hypothesis  is 
worth  considering. 

Treatment. — The  patient  should  be  put  as  quickly  as 
possible  in  a  quiet,  darkened  room.  Administer  calomel  at 
once,  in  a  sufficient  dose  to  effect  a  very  copious  discharge 
from  the  bowels.  If  the  child  will  tolerate  it,  put  an  ice-bag 
over  the  nape  of  the  neck ;  or,  if  the  ice-bag  is  unpleasant, 
ordinary  cold  applications  can  be  tried.  As  soon  as  the 
bowels  have  been  moved  give  the  iodides,  the  bromides, 
or  mercury ;  employ  those  therapeutic  measures  which  we 
have  discussed  in  connection  with  other  forms  of  menin- 
gitis. 

From  the  very  beginning,  too,  observe  the  cardiac  and 
respiratory  functions,  and  give  mild  cardiac  stimulants. 
The  best  are  small  doses  of  digitalis  and  caffeine. 

Special  attention  should  be  paid  to  the  feeding  of  the 
child.  Feeding  by  the  spoon  and  giving  the  food  in  very 
small  quantities  at  a  time  is  the  only  proper  method.  If 
great  care  is  not  exercised  the  liquids  may  flow  into  the 
trachea  and  produce  very  uncomfortable  symptoms,  with 
the  possible  result  of  complicating  pneumonia.  According 
to  the  condition  of  the  child  the  physician  should  exercise 
his  judgment,  and  remember  that  loss  of  sleep  and  lack  of 
quiet  are  often  much  more  harmful  than  lack  of  food,  and 
every  child  suffering  from  any  form  of  meningitis  should 
be  given  ample  time  for  quiet  sleep.  The  old  habit  of  in- 
sisting on  half-hourly,  or  even  hourly,  feeding  is  not  to  be 
commended  in  these  cases. 

Unfortunately  there  is  little  reason  with  the  majority  of 
patients  to  change  this  method  of  treatment,  for  whatever 
method  be  employed  the  results  are  equally  disastrous;  but 
a  persistent  effort  should  be  made,  and  the  attempt  to  con- 
quer the  disease  should  not  be  given  up  until  the  child  be- 
gins to  decline  rapidly,  until  it  fails  to  swallow  food,  for 


404  THE  NERVOUS  DISEASES   OF   CHILDREN. 

from  that  time  on  medication  will  do  very  little  good.  Per- 
sistent administration  of  medicines,  of  nutrient  enemata, 
and  the  like,  beneficial  as  they  may  be  in  other  diseases,  in 
these  simply  tend  to  prolong  the  agony  of  the  child,  and  of 
the  careworn  parents  or  relatives.  Lumbar  puncture  is  of 
no  avail  in  this  form  of  meningitis,  and,  after  the  diagnosis 
has  been  established,  should  not  be  repeated. 

Surgical  interference  has  been  attempted  in  tubercular  meningitis.  Ord 
and  Waterhouse  have  trephined  a  case  diagnosticated  as  tubercular  menin- 
gitis, and  have  drained  the  subarachnoid  space ;  *  the  child  recovered.  There 
is  reason  to  doubt  the  tubercular  character  of  the  disease  in  this  case,  but  the 
relief  after  the  operation  was  so  marked  that  the  propriety  of  an  operation 
can  be  entertained  before  the  child  is  exhausted,  if  pressure  symptoms  are 
extreme  and  the  character  of  the  meningitis  is  doubtful.  A  recent  author 
(Hirschberg)  believes  that  death  is  due,  not  to  the  tubercles,  but  to  intracra- 
nial compression.  We  have  stated  on  a  preceding  page  that  the  increase  in 
intracranial  fluid  is  often  very  slight  indeed.  The  trephine  in  Ord's  patient 
was  applied  midway  between  the  external  occipital  crest  and  mastoid  process. 
The  operation  has  been  attempted  in  this  city  on  an  undoubted  case  of  tuber- 
cular meningitis  and  the  child  died  very  shortly  thereafter. 


EPIDEMIC   CEREBRO-SPINAL   MENINGITIS.f 

This  special  form  of  meningitis  has  attained  a  sad  dis- 
tinction in  many  countries.  In  America  its  ravages  have 
been  very  much  greater  than  in  Europe.;}:  In  New  York 
City  serious  epidemics  occurred  in  the  winter  and  spring 
of  1904  and  1905.  It  belongs  to  the  category  of  infectious 
fevers.  The  microbic  origin  of  the  disease  has  recently 
been  established  beyond  peradventure,  though  it  seems  to 
be  still  a  matter  of  doubt  as  to  whether  one  or  more  micro- 
organisms bear  an  etiological  relation  to  it. 

*  Such  drainage  could  be  effected  quite  as  readily  by  a  lumbar  puncture  according 
to  Quincke's  method. 

t  Synonyms:  "  Spotted  fever,"  "  cerebro-spinal  fever,"  "  typhus  petechialis,"  "  febris 
nigra,"  and  "malignant  meningitis,"  are  the  common  synonyms,  all  of  them  indicating 
the  infectious  and  grave  character  of  the  disease 

%  At  this  present  time  (1905)  serious  epidemics  of  "  spotted  fever  "  have  been  reported 
from  various  parts  of  Continental  Europe.  In  eastern  Prussia  the  disease  appeared  in 
its  most  virulent  form  ;  and  if  the  earliest  statistics  are  reliable,  the  mortality  rate  is 
about  the  same  as  in  American  cities.  In  spite  of  "  commissions  "  that  have  been  ap- 
pointed, no  sovereign  remedy  has  been  discovered. 


MENINGITIS.  405 

It  is  the  accepted  opinion  of  the  day  that  this  grave  disease  is  due  to 
infection  by  the  diplococcus  intracellularis  (Weichselbaum,  Jaeger,  Hcubner, 
Osier,  Koplik,  Holt,  and  others).  Some  sporadic  cases  have  been  shown  to 
be  due  to  the  micrococcus  pneumonia;  of  Fraenkel,  and  in  some  cases, 
resembling  the  epidemic  form  clinically,  streptococci  and  other  micro- 
organisms have  been  found.  The  only  just  inference  is,  that  various  or- 
ganisms may  produce  cerebro-spinal  meningitis,  but  that  the  epidemic  form, 
and  no  doubt  many  of  the  sporadic  cases,  are  due  to  the  diplococcus  intra- 
cellularis. 

The  epidemics  have  varied  much  in  severity,  and  in  each  epidemic  there 
have  been  some  mild,  and  an  alarming  number  of  most  virulent  cases.  The 
micro-organisms  have  been  found  in  the  nasal  secretions,  thus  suggesting  a 
possible  point  of  entrance  into  the  cranial  cavity.  Among  the  New  York 
physicians  reporting  upon  the  latest  epidemics  are  Chapin,  Nammack,  Manges, 
Koplik,  Lambert,  Berg,  and  others. 

The  first  epidemic  of  cerebro-spinal  meningitis  was  distinctly  recognized 
as  such,  and  well  described  under  the  heading  of  "  a  malignant  non-conta- 
gious fever  "  by  Vieussens  in  1805,  who  described  the  disease  as  it  appeared 
in  Geneva.  Thirty-three  persons  lost  their  lives  during  this  epidemic  ;  the 
average  duration  of  the  disease  was  from  one-half  to  five  days.  A  few 
years  later  epidemics  occurred  in  various  parts  of  Germany,  in  Holland,  and 
in  England.  Dr.  J.  Lewis  Smith,  to  whom  we  are  indebted  for  one  of  the 
best  contributions  to  our  knowledge  of  this  disease,  states  that  the  first  Amer- 
ican case  occurred  at  Medfield,  Mass.,  in  1806.  From  1806  to  1816  it  ap- 
peared in  various  localities  both  in  Canada  and  in  the  Linked  States.  Be- 
tween 1 8 16  and  1828  one  epidemic  occurred  at  Middletown,  Conn.,  and 
another  at  Vesoul,  in  France.  In  1833  Naples  was  visited  by  this  epidemic, 
and  the  disease  did  not  appear  again  until  1837,  and  then  various  localities  in 
France  were  stricken.  The  military  were  chiefly  affected  by  the  disease,  and 
a  very  large  proportion  of  those  affected  died  from  it.  Between  1837  and 
1849  France  was  the  chief  seat  of  the  epidemic.  In  the  next  ten  years  al- 
most every  part  of  Europe  was  visited  by  the  disease.  In  1842  another  epi- 
demic broke  out  in  the  United  States,  at  a  distance  from  the  sea-coast,  and  as 
Dr.  Smith  says,  apparently  not  by  communication  from  Europe  ;  but  this 
could  hardly  be  maintained  with  our  present  views  regarding  the  transmis- 
sion of  micro-organisms  from  one  country  to  another.  Epidemics  occurred 
in  States  as  widely  apart  as  Alabama  and  Mississippi,  New  York  and  Louis- 
iana. Norway  and  Sweden  were  the  chief  seats  of  the  disease  between  1854 
and  i860,  and  since  that  time  scarcely  a  single  city  or  district  has  been 
entirely  free  from  the  disease.  Inasmuch  as  isolated  districts  have  been  af- 
fected, the  disease  was  not  supposed  to  spread  in  the  manner  of  ordinary 
contagious  diseases,  but  to  have  been  engendered  by  local  conditions,  among 
which  the  massing  together  of  large  classes  of  population  in  poorly  ventilated 
and  filthy  quarters,  as  in  military  barracks  for  instance,  was  considered  to 
be  the  most  favorable  predisposing  cause.  The  disease  is  now  perma- 
nently established  in  almost  every  large  American  city,  though  it  has  rarely 
assumed  a  severe  epidemic  form.     In  New  York  City,  from  1866  to  1872,  the 


406 


THE  NERVOUS  DISEASES   OF  CHILDREN. 


annual  deaths  from  this  disease,  according  to  Dr.  Smith's  statistics,  varied 
from  eighteen  to  forty-eight.  A  very  severe  epidemic  occurred  in  December, 
1 87 1,  and  lasted  well  into  the  summer  of  1872,  so"  that  782  deaths,  chiefly  in 
children,  resulted  from  cerebro-spinal  fever  in  this  city  alone.  Since  that 
time  the  annual  deaths  have  varied  between  97,  in  1878,  and  461,  in  1881. 
An  important  epidemic,  though  a  small  one,  was  the  epidemic  of  Lonaconing, 
which  was  most  carefully  observed  by  two  competent  physicians,  of  which 
an  account  is  given  in  the  article  by  Flexner  and  Barker  in  the  year  1894. 
This  town  is  situated  in  the  Alleghany  Mountains  and  contains  about  five 
thousand  inhabitants.  A  muddy  stream  which  passes  through  the  town, 
receiving  most  of  the  sewage,  appears  to  have  been  responsible  for  the  spread- 
ing of  the  epidemic.  All  the  conditions,  including  the  overcrowding  of 
miners  in  filthy  houses,  were  favorable  to  the  spread  of  the  disease. 

The  disease  is  common  both  to  man  and  beast.  A  serious  epidemic 
occurred  in  New  York  City  in  1871,  and  was  at  once  recognized  as  a  filth 
disease,  for  it  first  affected  the  horses  in  the  large  and  overcrowded  stables 
of  the  car  and  stage  lines.  A  few  individuals  were  soon  similarly  afflicted, 
but  it  is  doubtful  whether  the  disease  was  transmitted  from  the  animals  to 
the  men  who  were  in  charge  of  them.  The  epidemic  which  occurred  in  1872 
was  evidently  related  to  this  same  outbreak  in  animals  in  1871.  Though 
the  disease  is  bred  by  filth,  it  may  unquestionably  also  be  carried,  or  at  least 
transmitted,  to  persons,  and  particularly  to  children  living  in  excellent  hy- 
gienic surroundings,  and  many  of  us  have  seen  such  cases  in  the  habitations 
of  the  richest  as  well  as  in  the  families  of  the  working-classes.  The  disease 
may  attack  those  in  good  health,  but  is  even  more  apt  to  strike  those  whose 
health  has  been  injured  by  previous  disease  or  by  fatigue.  Dr.  Smith  quotes 
Frothingham  as  an  authority  for  the  statement  that  in  a  brigade  of  the  Army 
of  the  Potomac  which  was  attacked  by  this  epidemic,  the  men  were  almost 
exhausted  from  excessive  drilling. 

The  disease  shows  no  distinction  between  the  sexes,  at  least  in  children. 
Dr.  Smith  reports  105  cases  occurring  in  his  practice,  of  which  59  were  in  males 
and  46  in  females  ;  91  of  these  cases  being  in  children.  While  persons  of 
every  age  may  be  attacked  by  the  disease,  it  is  unquestionably  more  liable  to 
attack  children  in  the  earlier  years  of  life  than  at  any  other  period.  It  is  in- 
teresting to  note  the  statistics  for  a  single  year,  1883,  as  given  by  Smith  for 
New  York  City : 


Under 

From 

1  to 

2 

years 

From 

2  to 

3 

« 

From 

3  to 

4 

" 

From 

4  to 

5 

" 

From 

5  to 

10 

" 

From  10  to 

15 

" 

57 
3i 
22 
12 

9 

37 
18 


From  1 5  to  20  years 15 

From  20  to  25     "     

From  25  to  30     ""    

From  30  to  35     "     

From  35  to  40     "     

After  this  scattering  cases. 


The  New  York  Health  Department  records  498  deaths  from  cerebro-spinal 
meningitis  from  March  12th  to  May  21st,  1904. 


MENINGITIS.  407 

Symptoms. — The  majority  of  the  symptoms  will  natu- 
rally resemble  those  occurring  in  other  forms  of  meningitis. 
The  onset  of  the  disease  is  characteristically  sudden.  A 
child  in  perfect  health  is  suddenly  seized  with  headache, 
vomiting,  and  either  slight  rigor  or  convulsions.  In  the 
milder  cases  a  few  days  of  malaise  and  of  slight  nausea  may 
precede  the  onset  of  the  other  symptoms.  The  first  severe 
symptoms  are  promptly  followed  by  a  stupor,  which  is  apt 
soon  to  deepen  into  profound  coma.  I  remember  the  case 
of  a  child,  one  year  of  age,  in  which  a  slight  headache, 
vomiting,  convulsions,  rigidity  of  the  neck,  deep  coma,  and 
strabismus  all  developed  within  twenty-four  hours,  and 
death  ensued  within  forty-eight.  Severe  neuralgic  pains 
are  frequent  in  the  earlier  stages  of  the  disease.  The  pupils 
are  unequal  and  dilated.  The  vomiting  is  of  the  cerebral 
order  and  occurs  on  the  first  or  the  second  day  in  the 
vast  majority  of  the  cases.  The  fever  is,  as  a  rule,  high, 
varying  between  1030  and  1050  F. ;  in  one  rapidly  fatal  case 
it  reached  1070  F.  a  few  hours  before  death  on  the  second 
day. 

The  meningeal  symptoms  are  developed  in  a  very  much 
shorter  period  of  time  than  in  other  forms  of  meningitis.  We 
do  not  have  the  slow  progress  from  stupor  to  coma  which 
we  find  in  the  tubercular  type ;  often  the  coma  is  deep 
from  the  very  beginning.  Delirium  may  alternate  with  in- 
tense coma.  In  some  of  the  cases  in  which  the  coma  is  not 
profound,  great  restlessness  takes  the  place  of  the  stuporous 
condition.  The  child  is,  as  a  rule,  extremely  sensitive,  even 
hyperaesthetic.  The  slightest  touch  of  any  part  of  the  body, 
the  mere  weight  of  the  bedclothes,  is  often  sufficient  to 
elicit  shrill  cries.  This  hypersesthesia  is  explained  quite 
readily  by  the  irritation  of  all  the  posterior  root-fibres  by 
the  meningeal  exudate.  Contractions  of  the  various  mus- 
cles occur  at  an  early  stage  of  the  disease.  The  head  is 
firmly  retracted,  opisthotonos  is  distinctly  developed,  the 
thighs  and  legs  are  in  a  flexed  position,  and  the  arms  and 
hands  may  be  distinctly  contractured.  A  hemiplegia  or  an 
alternate  form  of  paralysis,  or  a  monoplegia,  together  with 
cranial  nerve  palsies,  may  be  made  out  in  some  patients. 
The  deep   reflexes   are   exaggerated,    and   the    superficial 


408  THE   NERVOUS  DISEASES   OF  CHILDREN. 

reflexes  are  diminished.     Kernig's  sign  is  present.     Convul- 
sions are  very  common. 

The  author  saw  during  the  spring  months  of  1904  a  number  of  fulminant 
cases  in  which  the  disease  ran  its  entire  course  within  forty-eight  hours.  In 
such  cases  the  chief  symptoms  are  :  Headaches,  high  fever,  rapidly  developing 
delirium,  rigidity  of  the  neck  ;  in  short,  general,  not  localizing,  symptoms. 

The  "  tache  cerebrale  "  was  formerly  considered  to  be  a 
rather  important  symptom  of  all  forms  of  meningitis ;  but 
it  has  lost  its  pathognomonic  value,  as  it  occurs  in  many 
other  diseases.  The  skin  presents  a  peculiar  mottling  in 
the  first  or  second  week  of  the  disease,  and  particularly 
when  the  temperature  is  low.  Small  red  points  and  large 
bluish  spots,  due  to  exudation  of  blood  under  the  surface, 
also  appear,  and  were  seen  frequently  enough  to  justify  the 
term  "  spotted  fever."  In  European  epidemics  these  peculiar 
extravasations  have  not  been  regularly  observed.  Herpes 
occurs,  and  Smith  refers  to  the  occurrence  of  erysipelas ; 
but  the  latter  is  evidently  entirely  independent  of  the  men- 
ingitis; it  is  a  complicating  condition,  and  not  in  any  sense 
a  symptom  of  the  epidemic  form. 

The  organs  of  special  senses  are  frequently  affected  in  epidemic  menin- 
gitis, and  possibly  more  frequently  than  in  other  forms.  A  hypersemic  and 
inflammatory  condition  of  the  entire  eyeball  is  a  common  occurrence.  The 
media  may  become  cloudy  and  the  various  structures  may  become  adherent 
to  one  another  ;  occasionally  ulcerations  of  the  cornea  and  perforation  of  the 
eye  with  total  loss  of  vision  may  occur.  According  to  Knapp,  as  quoted  by 
Smith,  the  nature  of  the  eye  affection  is  a  purulent  choroiditis,  probably  met- 
astatic. In  some  cases  a  double  optic  neuritis  occurs,  and  from  this,  as  well 
as  from  the  inflammatory  conditions  of  the  eyeball,  total  blindness  may  re- 
sult. Not  a  few  of  those  who  recover  from  cerebro-spinal  meningitis  are 
afflicted  with  permanent  blindness  as  the  result  of  this  dreadful  scourge. 

The  hearing  is  often  as  seriously  impaired  as  vision,  and  severe  otitis 
media,  ending  in  suppuration,  with  perforation  of  the  drum  and  all  its  se- 
quences, is  a  common  occurrence.  In  other  instances  loss  of  hearing  is  evi- 
dently due  to  more  central  causes,  and  is  developed  only  after  recovery  from 
the  main  disease.  It  is  unfortunate  that  the  loss  of  hearing  is  apt  to  be  bi- 
lateral and  complete.  According  to  Smith's  statistics  from  the  epidemic  in 
1872,  about  one  in  every  ten  patients  became  deaf.  But  he  states  that  in  the 
milder  form  of  cerebro-spinal  meningitis  which  has  prevailed  since  1872  the 
proportionate  number  that  has  been  thus  affected  has  been  less,  and  the 
same  may  be  said  with  reference  to  loss  of   sight.      Knapp  reports  that 


MENINGITIS.  409 

among  twenty-nine  cases  of  total  deafness  occurring  after  cerebro-spinal 
meningitis  only  one  seemed  to  give  evidence  of  hearing  afterward. 

Morbid  Anatomy  and  Pathology. — We  need  not 
again  insist  upon  the  microbic  origin  of  the  disease.  If  the 
exudate  is  examined  the  diplococcus  intracellularis  or  the 
pneumococcus  may  be  found.  The  chief  anatomical  charac- 
teristics of  the  disease  are  an  intense  hyperaemic  condition  of 
the  meninges  and  of  the  brain,  and  this  may  be  the  sole 
morbid  condition  if  the  patient  has  died  in  the  very  early 
stage  of  the  disease.  If  it  has  lasted  more  than  a  few 
days,  pus  is  visible  to  the  naked  eye  under  the  arachnoid. 
This  membrane  loses  its  transparency  and  begins  to  appear 
cloudy,  the  cloudiness  being  most  apparent  along  the  course 
of  the  vessels  from  which  the  exudation  undoubtedly  takes 
place. 

The  pus  is  found  both  in  the  meshes  of  the  pia,  and  un- 
der the  pia,  between  it  and  the  cortex.*  The  fibrinous  puru- 
lent layer  will  be  found  adherent  to  the  pia,  and  can  usually 
be  removed  together  with  this  membrane.  These  purulent 
layers  can  be  drawn  out  of  the  fissures,  leaving  discolored 
tissue  underneath.  This  purulent  exudation  covers  not 
only  the  fissures  of  the  convexity,  but  extends  with  equal 
frequency  over  the  base  of  the  brain,  and  in  those  very 
spaces  in  which  we  are  accustomed  to  look  for  tubercular 
deposits.  In  addition  to  the  exudation  over  the  brain 
proper,  an  equally  thick  layer  can  often  be  found  over  the 
greater  part  of  the  spinal  cord,  holding  the  same  relations  to 
the  spinal  meninges  that  it  does  to  the  cerebral  coverings. 
The  blood  is  apt  to  be  clotted  in  the  large  veins  and  sinuses, 
and  such  clots  may  be  of  a  purulent  character.  The  ventricu- 
lar fluid  is,  as  a  rule,  increased,  and  in  the  more  violent  forms 
may  contain  small  floccules  of  fibrin  or  fibrinous  pus. 

In  addition  we  may  find  hypostatic  pneumonia,  or  varying  degrees  of 
bronchitis  and  atelectasis  ;  all  the  serous  membranes  may  be  in  a  condition 
of  inflammation.  The  spleen  is  almost  invariably  enlarged,  while  the  other 
abdominal  viscera  are  found  in  a  condition  of  decided  hyperaemia ;  the  kid- 
neys may  be  in  a  condition  of  acute  congestion,  and,  according  to  Welch,  an 
acute  diffuse  nephritis  is  occasionally  present. 

*  An  excellent  colored  plate  of  a  purulent  meningitis  may  be  found  in  Holt's  text- 
book, page  752. 


410  THE  NERVOUS  DISEASES   OF   CHILDREN. 

Differential  Diagnosis. — The  only  point  to  be  con- 
sidered in  this  respect  is  the  differentiation  of  this  form 
from  other  forms  of  meningitis.  This  differentiation  should 
not  depend  solely  upon  the  bacteriologic  findings,  but  note 
that  the  disease  advances  much  more  rapidly  than  do  the 
tubercular  or  other  types.  The  mental  symptoms  are,  above 
all  things,  developed  much  more  actively  and  vehemently, 
in  keeping  with  the  early  exudation  of  pus  over  the  surface 
of  the  brain.  The  temperature  is  higher  in  the  earliest 
period  of  the  disease.  Cranial  nerve  palsies  are  often  want- 
ing. The  occurrence  of  similar  disease  in  the  city,  the  con- 
dition of  the  environment  of  the  patient,  and  the  exclusion  of 
those  facts  which  tend  to  prove  the  presence  of  tubercular 
disease  will  help  to  make  the  diagnosis  of  the  epidemic  form 
much  more  certain. 

Prognosis. — The  disease  is  unquestionably  one  of  the 
most  fatal  diseases  of  childhood,  and  if  it  does  not  prove 
fatal  the  condition  of  the  survivor  is  often  so  distressing 
that  death  would  have  been  preferable.  About  one-half  of 
the  patients  make  a  fair  recovery.  The  duration  of  the 
coma  is  as  reliable  a  sign  as  any  in  giving  a  prognosis. 
Cases  in  which  the  coma  is  rapidly  developed  and  does  not 
show  any  sign  of  receding  within  the  first  week  or  two  are 
almost  certain  to  end  fatally.  If  the  coma  has  been  slow  to 
develop,  the  inference  is  justified  that  the  process  is  a  less 
intense  one,  and  there  is  in  so  far  a  hope  of  recovery.  But 
even  in  these  cases  if  the  coma  which  has  once  been  devel- 
oped remains  stationary  for  a  week  or  more,  the  chances  of 
recovery  lessen  with  almost  every  hour  that  the  coma  con- 
tinues. In  spite  of  all  rules  that  may  be  laid  down  children 
whom  we  have  reason  to  expect  to  recover  take  a  turn  for 
the  worse,  and  not  a  few  of  those  who  have  been  given 
up  by  the  most  careful  and  experienced  physicians  make 
good  recoveries ;  but  in  others,  in  whom  the  disease  drags 
along,  the  final  issue  is  simply  deferred,  and  death  may 
result  from  exhaustion,  as  in  one  of  my  own  cases,  as  late 
as  four  months  after  the  onset  of  the  disease. 

Treatment. — The  treatment  of  this  special  form  of 
meningitis  can  differ  in  no  respect  from  that  noted  in  con- 
nection with  the  discussion  of  the  tubercular   and  simple 


MENINGITIS.  41  I 

forms  of  meningitis.  Although  the  disease  has  not  been 
proved  to  be  contagious,  the  patient  should  be  strictly 
isolated. 

In  the  epidemic  of  1904,  Manges,  following  the  recommendation  of  Seager, 
who  reported  upon  an  epidemic  in  Lisbon,  advocated  the  use  of  a  three 
to  nine  c.  c.  injection  of  a  one  per  cent.  Lysol  solution  into  the  spinal  canal 
after  the  withdrawal  of  cerebro-spinal  fluid.  Three  cases,  treated  in  this 
fashion  (one  of  them  a  streptococcus  infection)  recovered.  In  the  hands 
of  others  this  treatment  has  failed.  During  the  present  epidemic,  the  sug- 
gestion has  been  made  to  use  the  Diphtheria  Antitoxin  in  the  treatment  of 
epidemic  cerebro-spinal  meningitis.  If  such  treatment  prove  successful,  the 
Bacteriologists  will  have  to  revise  their  fundamental  doctrines  and  beliefs. 

MENINGITIS   DUE   TO   OTHER   CAUSES.* 

I.  Meningitis  due  to  traumatism  has  been  mentioned  in 
connection  with  acute  meningitis ;  it  is  much  rarer  in  chil- 
dren than  in  adults,  for  reasons  which  it  is  hardly  necessary 
to  explain;  though  the  injuries  which  children  receive  may 
be  relatively  slight,  meningeal  disturbances  follow  upon 
them  in  some  instances.  A  fall  from  a  chair,  a  fall  down 
the  stairs,  or  a  blow  dealt  upon  the  head  of  the  child,  to 
which  little  heed  is  paid  at  the  time,  may  be  the  starting- 
point  of  the  meningitis.  At  times  the  same  slight  traumatic 
factor  is  the  cause  of  an  epilepsy  developing  after  six 
months,  or  even  after  a  year,  or  still  later  (see  chapter  on 
Epilepsy).  In  these  children  a  local  meningitis  or  meningo- 
encephalitis may  be  considered  to  be  the  actual  lesion  re- 
sponsible for  the  epilepsy.  With  such  cases  we  have  no 
concern  at  present.  We  must  take  into  account,  however, 
those  patients  in  whom  some  traumatism  to  the  skull  is  fol- 
lowed by  the  symptoms  characteristic  of  meningitis.  The 
inflammatory  process  may  involve  both  the  dura  and  the 
pia,  and  may  be  either  serous  or  purulent. 

In  these  days  of  cranial  surgery,  another  form  of  (purulent)  meningitis  is 
to  be  observed  in  children  and  in  adults  after  operative  procedures  for  the  re- 
lief of  epilepsy  or  other  br^in  diseases.  The  aseptic  principles  of  modern  sur- 
gery are  calculated  to  prevent  such  complications.  It  is  exceptional  to  see 
this  condition  in  cases  in  which  a  sirnple  trephining  operation  has  been  done, 
but  if  the  dura  has  been  opened  the  danger  of  meningitis  is  greater. 

*  For  "  Meningitis  serosa"  see  page  421. 


412  THE   NERVOUS  DISEASES   OF   CHILDREN. 

The  prognosis  of  a  meningitis  developing  after  opera- 
tion is,  on  the  whole,  excessively  grave.  The  only  advice 
to  be  given  is  that  as  soon  as  the  temperature  rises,  and  as 
soon  as  the  first  symptoms  of  a  suppurative  meningitis  set 
in,  the  wound  should  be  opened  at  once,  and  an  effort  made 
to  treat  the  condition  according  to  the  best  surgical  prin- 
ciples. The  same  applies  to  those  cases  of  meningitis  which 
follow  upon  external  injuries,  if  the  meningeal  symptoms 
can  be  clearly  traced  to  the  preceding  injury.  A  careful 
examination  of  the  skull  should  be  made  in  order  to  deter- 
mine, if  possible,  where  the  injury  has  been  inflicted.  If 
the  slightest  depression  can  be  felt,  trephining  should  be 
done  over  this  region,  and  if  no  changes  in  the  skull  can  be 
made  out,  but  the  symptoms  point  to  an  incipient  meningitis, 
any  abrasion  of  the  scalp,  or  any  extravasation  of  blood  un- 
der the  scalp,  should  be  a  sufficient  guide  as  to  the  site  of 
the  operation.  1  am  firmly  convinced  that  much  good  can 
be  done  by  proper  and  timely  surgical  interference  in  these 
cases,  and  little  harm  will  result  even  if  the  operation  should 
prove  that  no  tangible  local  injury  has  been  done  to  the 
brain  or  skull.  To  be  sure  the  operation  should  not  be  un- 
dertaken if  the  child's  general  condition  is  such  that  it  will 
not  bear  the  shock  of  the  operation.  In  such  circumstances 
the  calm  judgment  of  an  experienced  surgeon  or  physician 
will  be  of  great  value,  but  it  should  be  distinctly  stated  that 
coma,  however  profound,  or  recurrent  convulsions,  do  not 
constitute  a  contra-indication  to  operative  interference.* 

II.  Meningitis  in  a  purulent  form  may  result  from  dis- 
ease of  the  ear.  The  aurists  are  well  aware  of  the  danger 
lurking  in  every  form  of  disease  of  the  mastoid  and  of  the 
inner  or  middle  ear ;  for  if  the  pus  that  is  formed  in  any  one 
of  these  regions  is  not  discharged  outward,  the  danger  of  its 
causing  a  purulent  meningitis  by  a  direct  discharge  inward, 
or  through  caries  of  the  petrous  bone,  is  much  to  be  feared. 
Chronic  ear  trouble,  so  slight  that  little  attention  is  paid 
to  it,  may  persist  for  years  before  causing  a  purulent  men- 

*  Too  much  valuable  time  is  wasted  with  the  administration  of  drugs  that  may  ease 
the  physician's  conscience  but  do  the  patient  little  or  no  good.  If  the  symptoms  point 
to  an  increase  of  intracranial  pressure,  or  to  an  extension  of  the  morbid  process,  an 
exploratory  trephining  should  be  attempted. 


MENINGITIS.  4 1  3 

ingitis.*  Since  aural  surgeons  have  become  accustomed 
to  operate  promptly  upon  the  appearance  of  symptoms 
pointing  to  suppuration  in  the  mastoid  process,  or  in  the 
other  bony  parts  of  the  ear,  these  forms  of  purulent  menin- 
gitis from  ear  disease  are  far  less  frequent  than  they  were 
formerly.  The  symptoms  which  point  to  an  incipient 
meningitis  can  be  distinguished  in  many  cases,  though  not 
in  all,  from  those  due  to  the  presence  of  pus  within  the  ear 
structures  alone.  In  addition  to  the  intense  pain,  the  pres- 
ence of  pus  in  the  ear  may  cause  giddiness,  intense  vertigo, 
vomiting,  and  even  slight  stupor,  but  if  the  headaches  be- 
come general  and  most  intense,  if  the  child  becomes  coma- 
tose, if  the  pulse  is  either  slowed  up  or  very  much  acceler- 
ated, if  the  neck  becomes  sensitive  and  rigid,  and  if  a  slight 
optic  neuritis  should  set  in  in  one  or  both  eyes,  it  is  certain 
that  the  pus  has  passed  beyond  the  limits  of  the  ear  and  has 
set  up  a  meningitis,  or  possibly  an  abscess.  Under  such 
conditions  it  is  imperative  to  operate,  giving  the  pus  a 
chance  to  discharge  outward,  and  to  lay  bare,  if  necessary, 
the  parts  of  the  brain  which  are  most  apt  to  be  involved  in 
ear  disease.  It  will  be  advisable  at  the  start  thoroughly  to 
explore  the  mastoid,  as  well  as  the  middle  and  inner  ear,  be- 
fore attempting  to  trephine  over  the  temporal  convolutions, 
but  if  the  operations  upon  the  bony  parts  of  the  ear  do  not 
give  prompt  relief  the  cranial  operation  should  be  done.  The 
aural  surgeons  are  opposed  to  operations  in  cases  of  purulent 
meningitis,  but  as  long  as  the  meningitis  is  strictly  localized 
and  confined  to  the  parts  in  immediate  juxtaposition  to  the 
ear,  there  is  every  reason  to  advise  an  exploratory  operation 
to  secure  an  exit  for  the  pus,  which,  if  confined  to  these 
parts,  is  bound  to  cause  a  general  suppurative  meningitis. 

If  suppurative  meningitis  is  due  to  disease  of  the  mas- 
toid, the  pus  is  more  likely  to  find  its  way  either  to  the  cere- 
bellum or  to  the  base  of  the  brain.  In  diseases  of  the  middle 
and  inner  ear,  the  meningitis  is  more  apt  to  be  developed 


*  The  author  saw  this  well  illustrated  in  a  lad  of  fourteen  (a  patient  of  Dr.  Wiener), 
who  since  his  fourth  year  had  had  chronic  ear  disease  ;  without  special  cause  this  old 
trouble  was  lighted  up  and  ended  in  death  within  one  week.  The  autopsy  disclosed  a 
wide-spread  purulent  meningitis  from  caries  of  the  right  petrous  bone.  The  morbid 
condition  was  most  marked  over  the  left  temporo-sphenoidal  lobe. 


4H  THE  NERVOUS  DISEASES   OF   CHILDREN. 

in  the  regions  over  the  first  and  second  temporal  convolu- 
tions. In  endeavoring  to  locate  the  proper  region  for  open- 
ing the  skull  in  cases  of  ear  disease  it  is  best  to  select  a  point 
that  is  reached  by  going  one  and  a  quarter  inches  back  of 
the  external  auditory  meatus,  and  from  this  point  one  and  a 
quarter  inches  upward.  If  the  surgeon  remembers  that  a 
line  drawn  from  the  outer  angle  of  the  orbit  horizontally 
across  the  skull  gives  the  approximate  position  of  the  fissure 
of  Sylvius,  and  therefore  the  upper  limits  of  the  temporal 
convolutions,  he  cannot  well  fail  to  open  the  skull  over  the 
part  of  the  brain  that  comes  into  question  in  these  cases.* 

Meningitis  may  also  be  caused  by  a  tumor  or  an  abscess 
in  adjacent  parts  of  the  brain ;  but  in  such  cases  the  men- 
ingitis is  of  relatively  little  importance,  and  if  it  gives 
rise  to  distinct  symptoms  simply  helps  to  intensify  those 
caused  by  the  chief  morbid  process.  Purulent  meningitis  is 
sometimes  due  to  disease  of  the  nose,  to  erysipelas,  and  to 
purulent  disease  of  the  eye.  If  so,  the  antecedent  condi- 
tions will  leave  little  doubt  of  the  cause  of  the  meningitis, 
and  the  subsequent  symptoms  will  not  vary  from  the  men- 
ingitis following  upon  other  causes. 

III.  Many  of  the  infectious  diseases  of  children  lead  to 
meningitis.  Among  these  are  measles,  scarlatina,  small-pox, 
and  even  rheumatism  and  influenza,  but,  above  all,  typhoid 
fever  and  pneumonia. 

Meningitis  has  been  found  present  in  the  autopsies  upon 
persons  dead  from  typhoid  fever,  but  is  by  no  means  pres- 
ent in  all  those  forms  of  typhoid  fever  in  which  at  the  begin- 
ning or  in  the  later  stage  of  the  disease  delirium  and  coma 
are  developed  in  connection  with  the  fever.  If  a  child  have 
typhoid,  the  diagnosis  of  additional  meningitis  should  not 
be  made  unless  there  are  very  positive  symptoms,  such  as 
decided  rigidity  of  the  neck,  hyperesthesia  of  part  or  of  the 
entire  body,  and  cranial-nerve  palsies. 

Brain  symptoms  are  not  infrequent  complications  in 
acute  pneumonia,  and  much  attention  has  been  directed  to 
these  cerebral  complications  by  Holt  and  others;  but  menin- 
geal disease  unquestionably  occurs  in  a  number  of  instances, 
and  we  can  the  more  readily  understand  the  connection 

*  See  chapter  on  "  Abscess." 


MENINGITIS.  415 

between  the  two  diseases  since  Fraenkel's  pneumococcus 
has  been  found  in  the  inflamed  lung,  and  in  the  meningeal 
exudate.  The  same  coccus  is  also  found  at  times  in  the  epi- 
demic purulent  form  of  meningitis. 

IV.  Among  the  many  curious  and  surprising  sequelae 
of  influenza  which  have  been  described,  none  has  been  more 
striking  than  the  occasional  occurrence  of  cerebral  and  cere- 
brospinal meningitis  during  or  immediately  after  the  acute 
stage  of  the  disease.  For  the  present  the  evidence  is  in  favor 
of  an  encephalitis,  rather  than  a  meningitis,  as  the  cause  of 
this  special  form  of  disease.     (See  p.  416.) 

Inflammation  of  the  meninges  also  follows  upon  general 
septicemic  processes,  but  this  special  causation  is  much 
more  common  in  the  adult  than  in  the  child.  The  typical 
septicasmic  meningitis  is  that  form  which  we  encounter  in 
connection  with  the  puerperal  state  in  women,  and  from 
which  I  have  seen  one  most  remarkable  recovery.  There  is 
no  form  of  septicasmic  meningitis  in  children  quite  as  typical 
as  this  puerperal  meningitis,  unless  it  be  that  which  occasion- 
ally occurs  with  septic  bone  disease  or  with  ulcerative  endo- 
carditis. 

CHRONIC    BASILAR  MENINGITIS. 

A  special  form  of  meningitis  occurring  in  children  has  been  described  by- 
Gee,  Barlow,  Still,  and  others.  Its  only  claim  to  recognition  is  that  it  may 
run  a  protracted  course  varying  from  months  to  a  year  or  more,  that  it  affects 
very  young  children,  that  the  inflammation  is  most  marked  in  the  posterior 
fossa  of  the  base — and  that  for  a  long  time  cervical  opisthotonus  is  the  chief 
symptom.  Since  Still  has  shown  that  the  diplococcus  intracellularis  was 
found  in  seven  of  eight  cases,  and  since  these  cases  have  been  observed  dur- 
ing epidemics  of  cerebro-spinal  meningitis  (Koplik),  there  is  no  reason  to  re- 
gard chronic  basilar  meningitis  as  a  distinct  clinical  entity.  The  designation 
"  posterior  basic  meningitis  "  is  misleading ;  tuberculous  forms  are  "  posterior 
basic  "  often  enough,  as  well  as  anterior  basic,  or  middle  basic.  Such  terms 
produce  confusion. 

In  the  cases  so  classified,  lymph  may  glue  together  the  medulla  and  the  cere- 
bellum, the  openings  from  the  fourth  ventricle  may  be  closed,  and  the  ventricles 
distended.  The  writers  advocating  this  form  claim  that  the  symptoms  are: 
Gradual  or  sudden  onset,  convulsions,  slight  or  no  fever,  opisthotonus,  general 
muscular  rigidity,  mental  dulness  and  irritability,  rarely  coma.  We  agree 
with  Holt  that  some  forms  of  "  chronic  basilar  meningitis  "  in  children  may  be 
syphilitic.  As  for  treatment,  we  would  urge  lumbar  puncture,  to  be  repeated 
carefully  a  number  of  times,  and  the  administration  of  iodides,  about  ten  or 
fifteen  grains  per  day,  or  more — according  to  the  age  of  the  child. 


416  THE  NERVOUS  DISEASES   OF   CHILDREN. 

ACUTE   HEMORRHAGIC    ENCEPHALITIS. 

We  cannot  close  the  discussion  of  meningitis  without  reference  to  acute 
encephalitis,  which  is  frequently  associated  with  inflammation  of  the  pia,  but 
is  also  at  times  developed  independently  of  any  meningeal  trouble.  In  men- 
ingitis due  to  traumatism,  or  to  acute  and  chronic  intoxication,  some  enceph- 
alitis occurs  by  simple  extension  of  the  inflammation.  Striimpell,  Fiirbringer, 
Leichtenstern,  Oppenheim,  and  others  have  endeavored  to  establish  a  form  of 
acute  hemorrhagic  encephalitis  which  is  developed  commonly  after  some  acute 
infectious  disease,  particularly  after  influenza. 

Simple  acute  encephalitis  can  be  recognized  by  the  following  signs  :  The 
disease  is  very  apt  to  attack  children  before  the  age  of  puberty.  For  a  few 
days  previous  to  the  full  development  of  the  disease  the  child  complains  of 
headache,  of  dizziness,  and  is  irritable  or  depressed ;  drowsiness,  gradually 
developing  into  coma,  soon  sets  in.  Rigors  and  slight  elevations  of  temper- 
ature point  to  the  development  of  an  acute  infectious  trouble.  The  loss  of 
consciousness  need  not  be  complete,  and  distinct  remissions  occur  during  the 
first  week  of  the  disease.  Hours  of  stupor  are  followed  by  a  condition  of 
wakefulness  and  restlessness.  The  pupillary  reflexes  may  remain  normal  or 
may  be  sluggish ;  the  deep  and  superficial  reflexes  are  not  altered.  A  rigid- 
ity of  the  neck  and  slight  opisthotonos  are  early  symptoms.  Paralysis,  in 
the  form  of  monoplegia  or  a  hemiplegia,  is  observed  at  an  early  day,  and  may 
or  may  not  be  associated  with  aphasia.*  Ocular  and  other  cranial  nerve  pal- 
sies are  developed,  and  if  they  set  in  in  an  acute  fashion  the  condition  may 
remind  one  very  easily  of  a  basilar  meningitis,  with  the  exception  of  the  in- 
complete loss  of  consciousness.  Respiration  may  become  irregular,  and  the 
cardiac  action  is  either  accelerated,  irregular,  or  diminished.  Optic  neuritis 
has  been  observed  in  several  such  cases.  The  symptoms  will  vary  very  much 
according  to  the  seat  of  the  disease.  Loss  of  consciousness,  convulsions,  and 
palsies  will  be  more  frequent  in  encephalitis  of  the  convexity,  whereas  cranial 
nerve  palsies,  dizziness,  difficulties  in  deglutition  and  in  articulation  may  be 
expected  if  the  encephalitis  is  developed  chiefly  in  the  basilar  structures. 

The  cause  of  the  disease  will  vary  also  according  to  the  intensity  of  the 
encephalitis.  In  some  of  the  cases  the  coma  deepens,  the  patient  may  linger 
on  for  two  or  three  weeks,  but  finally  succumbs  to  the  disease,  the  entire 
course  reminding  one  very  much  of  the  pyaemic  forms  of  ncephalitis.  In 
other  patients,  after  the  disease  has  lasted  for  a  week  or  two,  prolonged 
remissions  and  complete  recoveries  may  set  in. 

As  an  instance  of  encephalitis  following  influenza,  I  wish  to  refer  to  the 
following  case,  although  the  possibility  of  a  meningitis  being  associated  with 
the  encephalitis  cannot  be  denied.  The  patient  and  several  other  members 
of  the  family  had  passed  through  the  usual  attacks  of  influenza  four  days 
previous  to  my  first  visit.  The  girl,  eighteen  years  of  age,  had  been  badly 
frightened  by  the  falling  of  a  picture.  That  same  evening  she  began  to 
vomit  and  complained  of  severe  headaches.     The  family  physician,  who  ex- 

*  On  the  relation  ol  the  acute  infantile  cerebral  palsies  acute  encephalitis,  cf.  Chap- 
ter XXV. 


MENINGITIS.  4 1 7 

amined  her,  found  a  temperature  of  1030  F.,  and  noticed  at  once  a  slight  re- 
traction of  the  neck.  For  the  next  few  days  the  temperature  varied  between 
1010  and  1030  F.  The  rigidity  of  the  neck  became  more  pronounced,  but 
the  pulse  and  respiration  were  not  sensibly  affected  at  any  time,  the  former 
being  at  the  rate  of  90  and  the  respirations  varying  between  16  and  20  a 
minute.  On  the  fourth  day  of  the  disease  there  was  a  very  marked  rigidity  of 
the  neck  and  great  pain  fulness  over  the  cervical  and  dorsal  spine  ;  excessive 
muscular  tenderness  in  the  lower  extremities,  such  as  is  frequently  found  in 
cases  of  simple  influenza.  The  deep  reflexes  were  lively,  but  not  excessively 
exaggerated.  The  patient  was  drowsy ;  if  left  to  herself  would  sleep,  but 
when  aroused  answered  all  questions  intelligently.  She  presented  in  addition 
a  marked  paralysis  of  the  rectus  externus  in  both  eyes.  There  was  loss  of  light 
reflexes  and  sluggishness  of  contraction  during  accommodation  in  both  eyes, 
but  there  were  no  other  third-nerve  symptoms.  The  papilla;  were  somewhat 
swollen.  These  symptoms  continued,  with  slight  changes,  for  two  weeks  ; 
then  she  began  to  improve  slowly ;  four  weeks  after  the  onset  of  the  first 
symptoms  the  patient  was  entirely  well. 

These  cases  surely  bear  a  strong  resemblance  to  the  ordinary  forms  of 
meningitis,  and  were  it  not  for  the  evidence  furnished  by  Fiirbringer,  Leyden, 
Eisenlohr,  and  Koenigsdorf  that  encephalitis  is  the  actual  condition,  the 
diagnosis  of  a  meningitis  could  be  defended  quite  as  readily. 

Morbid  Anatomy. — As  far  as  can  be  determined  at  the  present  time, 
the  morbid  process  is  a  simple  hemorrhagic  encephalitis.  The  inflammatory 
areas  are  small  and  strictly  circumscribed,  and  may  be  developed  in  symmet- 
rical parts  of  the  brain.  On  superficial  examination  the  brain  tissue  appears 
hyperasmic  and  dotted  with  small  red  points ;  the  areas  of  inflammation  are  a 
little  softer  to  the  touch  than  normal  tissue.  On  microscopical  examination 
the  smaller  vessels  are  found  dilated  to  their  utmost  capacity,  filled  with 
blood,  some  of  which  has  evidently  been  exuded  into  the  neighboring  tissue. 
Leucocytes,  granular  cells,  and  a  proliferation  of  the  cells  of  the  neuroglia 
complete  the  microscopical  picture.  There  may  be  slight  destruction  of  the 
nervous  elements,  but  this  will  depend  upon  the  intensity  of  the  process. 
The  entire  encephalitis  is,  no  doubt,  of  microbic  origin.  Southard  and  Keene 
have  very  recently  shown  that  it  may  be  due  to  the  staphylococcus  pyogenes 
aureus. 

The  prognosis  will  naturally  vary  according  to  the  intensity  of  the  symp- 
toms. A  fatal  issue  is  apt  to  follow  in  those  cases  in  which  the  symptoms, 
coming  on  with  great  violence,  point  to  an  intensely  septic  process.  If  the 
condition  of  coma  is  reached  by  slow  stages  and  remissions  are  observed 
within  the  first  week  or  ten  days,  a  favorable  turn  may  be  expected. 

The  treatment  will  consist  simply  in  absolute  quiet,  the  application  of  cold 
to  the  head  and  nape  of  the  neck,  and  in  the  administration  of  purgatives 
(calomel  is  by  far  the  best). 

Polio-encephalitis  Superior. — A  disease  due  to  the  inflammation 
of  the  gray  matter  of  the  floor  of  the  fourth  ventricle  and  of  the  aqueduct  of 
Sylvius,  had  been  described  by  Wernicke,  Thomsen,  and  others.  This  dis- 
ease has  been  observed  in  the  adult,  and  is  remarkably  frequent  in  persons 


4.i  8 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


presenting  the  symptoms  of  chronic  alcoholism.  This  special  form  would 
naturally  be  a  rarity  in  children  ;  the  symptoms  are,  however,  not  unlike  that 
form  of  encephalitis  which  has  been  described  in  connection  with  influenza. 
It  is  of  particular  interest,  also,  because  a  similar  condition  is  at  times  asso- 
ciated with  polio-myelitis.  We  have  often  referred  to  the  close  relation  be- 
tween the  gray  matter  in  the  spinal  cord  and  the  gray  matter  which  harbors 
the  nuclei  of  the  cranial  nerves.  We  can  readily  understand  why  the  same 
morbid  process  should  at  times  affect  the  cerebral,  and  at  other  times  the 
spinal  portion  of  this  central  gray  matter.     According  to  the  distribution  of 


Fig.  103. — Case  of  Unilateral  Nuclear  Palsy.  (Wiener.)  Hypoglossal  nucleus,  left 
side.  Photomicrograph  from  a  section  stained  after  Pal,  showing  .some  degenerated 
ganglion  cells. 

the  inflammation  in  the  region  of  the  ocular  nerve  nuclei,  or  in  the  vicinity  of 
the  nuclei  of  the  tenth  and  twelfth  nerves,  we  distinguish  between  polio- 
encephalitis superior  and  inferior.  The  disease  in  which  ocular  nerve  symp- 
toms (ophthalmoplegia,  partial  or  complete)  have  been  associated  with  polio- 
myelitis, is  termed  polio-encephalomyelitis.  This  form  has  been  described 
by  Rosenthal,  Seeligmuller,  Guinon,  myself,  and  others.  But  these  diseases 
are,  on  the  whole,  extremely  rare,  and  do  not,  as  a  rule,  begin  until  childhood 
is  past.     The  cases  also  take  a  more  or  less  subacute  course. 

Bulbar  Palsies.* — Diseases  of  the  pons  and  medulla  are  very  rare  in 
children ;  but  the  above  discussion  of  nuclear  diseases  leads  to  the  mention 

*  The  author  had  some  hesitation  in  discussing  these  diseases  in  this  chapter  ;  but 
they  are  too  rare  to  warrant  discussion  in  a  separate  chapter,  and  it  did  not  seem 
desirable  to  add  them  to  the  sections  on  congenital  nuclear  palsies. 


MENINGITIS. 


419 


of  progressive  bulbar  palsy.  This  disease  in  the  adult  bears  a  close  clinical 
and  anatomical  relation  to  progressive  amyotrophy  and  to  amyotrophic  I. 
sclerosis.  With  the  chronic  diseases  of  adults  we  are  not  concerned,  but  a 
short  allusion  may  be  made  to  progressive  bulbar  palsy  of  earlier  years, 
which  is  a  rare  disease,  but,  like  the  adult  form,  is  due  to  a  primary  degen- 
eration of  the  nuclei  of  the  lower  cranial  nerves.*  A  case  of  this  descrip- 
tion, from  myelinic,  was  carefully  studied  and  described  by  Dr.  Wiener. 

The  symptoms  of  unilateral  bulbar  palsy  were  discovered  quite  acciden- 
tally in  a  young  man  who  had  been  under  treatment  by  his  physician  for  tu- 
bercular glands  and  pharyngitis.  He  presented  :  1.  A  very  marked  deviation 
of  the  tongue  to  the  right  when  protruded.  2.  Distinct  atrophy  of  the  me- 
dian portion  of  the  right  half  of  the  tongue.     3.  The  faradaic  response  of 


Fig.  104.— Same  Case  as  Fig.  103.    Hypoglossal  nucleus,  right  side,  showing  absence 
and  extreme  degeneration  of  ganglion  cells. 


the  right  half  of  tongue  was  much  diminished  ;  the  contractions  were  slug- 
gish, there  was  increased  galvanic  excitability  of  the  rieht  side  (K.  C.  C.  < 
A.  C.  C.) ;  the  contractions  were  slow  and  wave-like.  4.  Taste  and  tactile  sen- 
sations were  normal.  5.  Deviation  of  the  soft  palate  and  of  the  uvula  to  the 
left  side.  On  phonation  the  paralysis  of  the  right  side  became  more  marked. 
6.  Great  difficulty  in  deglutition.  7.  Dysphonia  due  to  disease  of  the  right 
recurrent  laryngeal  nerve. 

Other  cranial  nerve  functions  were  normal.     All  reflexes  normal. 

A  gradual  progression  of  all  the  symptoms  led  to  a  fatal  issue  from  respira- 
tory failure.  Examination  of  the  brain  revealed  a  distinct  degeneration  of  the 
hypoglossal  and  vago-accessorius  nuclei  of  the  right  side,  with  degeneration 
of  the  respiratory  column.  In  the  hypoglossal  nucleus,  the  ganglion  cells 
were    greatly  changed  (atrophied,  shrunken,  and  granular).       The  ground 

*  No  special  reference  is  here  made  to  the  acute  and  the  pseudo-bulbar  palsies,  as 
both  these  conditions  are  very  rare  in  children  An  acute  polioencephalitis  inferior  in 
young  subjects,  giving  rise  to  bulbar  symptoms,  has  been  described. 


420  THE  NERVOUS  DISEASES   OF   CHILDREN. 

substance  did  not  stain  as  readily  in  the  right  as  in  the  left,  and  was  less 
compact ;  the  left  hypoglossal  nucleus  was  also  slightly  diseased  (lower  and 
outer  portions).     (See  Figs.  103,  104.) 

The  symptoms  of  bulbar  palsy  (paralysis  and  atrophy  of  the  muscles  of 
the  palate,  the  tongue,  the  pharynx,  and  the  larynx)  were  distinctly  present. 
In  some  subjects  there  is  additional  paralysis  of  the  lips  and  of  the  muscles 
of  mastication,  and  also  of  the  upper  divisions  of  the  facial  distribution. 
The  difficulties  of  deglutition  and  the  dysarthria  were  characteristic ;  all  these 
symptoms  justify  the  clinical  designation  of  glosso-labio-pharyngeal  paral- 
ysis. The  unilateral  character  of  the  symptoms  is  worthy  of  note,  but  does 
not  remove  the  case  here  recorded  from  the  category  of  subacute  or  chronic 
bulbar  palsies. 

The  disease  which  involves  the  nuclei  of  the  motor  cranial  nerves  is  occa- 
sionally associated  with  spastic  symptoms,  with  increase  of  the  deep  reflexes, 
and  with  atrophy  in  the  upper  extremities.  The  clinical  symptoms  thus  bear 
the  closest  resemblance  to  those  ofamyotrophic  lateral  scleras  is  and  the 
latter  disease  may  well  represent  an  extension  of  the  bulbar  process  into  the 
spinal  cord ;  the  spinal  symptoms  of  amyotrophic  lateral  sclerosis  may  pre- 
cede the  bulbar  symptoms  for  a  long  period  of  time.  All  these  forms  repre- 
sent a  disease  of  both  divisions  of  the  motor  tract. 

C.  H.  Brown  has  described  an  interesting  case  of  amyotrophic  lateral 
sclerosis  with  bulbar  symptoms  which  exhibited  the  first  signs  at  the  age  of 
twelve  years  or  earlier.  All  the  facial  muscles  were  involved  ;  the  boy  could 
not  laugh  or  swallow  easily ;  speech  was  nasal ;  there  was  atrophy  of  the 
sterno-cleido-mastoid  of  the  left  side,  and  to  a  lesser  extent  of  the  right. 
Other  neck  muscles  weak.  Considerable  atrophy  around  the  shoulder  gir- 
dle, of  all  of  the  arm  muscles,  and  of  the  interossei  and  the  thenar  group, 
fibrillary  twitchings  and  increased  reflexes  completed  the  series  of  symptoms. 
Progressive  bulbar  paralysis  in  children  has  also  been  described  by  Hoff- 
man, Remak,  and  Londe.  Eisenlohr  has  described  a  bulbar  form  of  infantile 
spinal  paralysis. 

Charcot,  Londe,  Brissaud,  Marie,  Hoffman  have  described  an  infantile, 
hereditary  or  family  form  of  progressive  bulbar  paralysis.  The  characteristic 
signs,  in  addition  to  the  bulbar  symptoms,  were  the  presence  of  stigmata  of 
degeneration,  the  involvement  of  the  upper  facial  branches,  the  occurrence  of 
ptosis  and  of  a  partial  or  complete  ophthalmoplegia. 

Oppenheim,  Peritz,  Bouchaud,  Ganghofner  have  described  an  infantile 
form  of  pseudobulbar  paralysis.  This  complex  of  symptoms  is  developed 
in  association  with  the  infantile  cerebral  palsies.  Glosso-labio  laryngeal 
symptoms  may  be  combined  with  spastic-athetoid  and  diplegic  symptoms. 
Oppenheim  and  Bouchaud  have  shown  this  form  to  be  due  to  defective  devel- 
opment (porencephaly)  of  the  lower  portion  of  the  central  convolutions. 


CHAPTER  XXIV. 
HYDROCEPHALUS. 

By  hydrocephalus  we  mean  an  excessive  accumulation 
of  serous  fluid  within  the  cranium,  either  in  the  subdural 
spaces  or  in  the  ventricles.  The  former  is  termed  exter- 
nal hydrocephalus ;  the  latter,  internal  hydrocephalus. 
It  is  convenient  also  to  divide  the  cases  into  acute  and 
chronic  forms,  and  to  subdivide  the  latter  into  congenital 
and  acquired  hydrocephalus.  As  the  accumulation  of  fluid 
often  follows  upon  other  diseases,  we  distinguish  a  form  of 
secondary  hydrocephalus  ;  the  term  primary  hydrocephalus 
remains  restricted  to  the  few  cases  in  which  the  accumu- 
lation of  fluid  appears  to  be  the  sole  morbid  condition. 
Chronic  hydrocephalus  is  by  far  the  more  important,  for 
what  might  be  said  of  truly  acute  hydrocephalus  has  already 
been  said  in  connection  with  the  primary  forms  of  meningi- 
tis ;  but  a  few  remarks  will  be  in  order,  at  this  juncture,  in 
relation  to  the  subject  of  acute  hydrocephalus,  neglecting 
for  a  time  the  hydrocephalic  condition  which  accompanies 
every  form  of  meningitis,  and  particularly  the  tubercular 
variety. 

Acute  Hydrocephalus.— Quincke,  in  an  able  article 
on  meningitis  serosa  states,  that  the  term  acute  hydrocepha- 
lus should  be  made  to  cover  the  condition  in  which  there  is 
a  purely  idiopathic  serous  meningeal  inflammation.  In  the 
cases  which  he  describes,  and  of  the  existence  of  which  there 
is  no  doubt,  the  symptoms  point  to  a  very  constant  increase 
of  intracranial  fluid  in  the  subpial  spaces  and  within  the 
ventricles.  Acute  hydrocephalus  is  characterized  generally 
by  very  sudden  onset;  occasionally  the  symptoms  come 
on  in  insidious  fashion.  Fever  may  be  entirely  wanting,  or, 
if  present,  rarely  exceeds  1030  F.  ;  headache,  rigidity  of  the 


422  THE  NERVOUS  DISEASES   OF  CHILDREN. 

neck,  nausea,  vomiting,  stupor,  coma,  delirium,  are  the  symp- 
toms present  in  this  disease,  and  they  are  the  same  which 
we  have  insisted  upon  so  frequently  in  connection  with  the 
various  forms  of  meningitis.  The  pupils  react  sluggishly, 
and  are  often  unequal  ;  optic  neuritis  is  present ;  convul- 
sions may  occur,  but  are  rare,  and  palsies  of  a  cerebral 
order  may  be  present,  but  are  rarely  either  severe  or  lasting. 
Paralysis  of  the  external  rectus  muscle  is  as  frequent  as  any, 
and  this  can  readily  be  explained  by  increased  intracranial 
pressure.  In  milder  cases  the  symptoms  soon  recede,  and 
absolute  recovery  may  take  place  within  a  few  weeks.  In 
the  more  serious  forms  the  symptoms  may  closely  resemble 
those  of  brain  tumor.  The  disturbance  of  function  due  to 
increased  intracranial  pressure  may  bring  about  a  fatal  issue. 
Some  of  these  types  of  meningitis  serosa  are  no  doubt  idio- 
pathic, others  may  represent  an  acute  exacerbation  of  a 
chronic  hydrocephalus.  These  diseases  occur  rarely  before 
the  first  year  of  life,  but  are  most  frequent  between  the  age  of 
one  and  five  years.  They  may  occur  up  to  the  age  of  thirty 
years,  and  even  later. 

Meningitis  serosa  may  be  due  either  to  traumatism  or 
to  acute  febrile  disease,  such  as  typhoid  and  pneumonia ;  in 
the  adult  the  excessive  use  of  alcohol,  and  the  pregnant 
state  may  be  responsible  for  the  development  of  the  disease. 
It  is  probable  that  this  serous  form  of  meningitis  is  not  of 
microbic  origin.  A  distinction  should  be  made  in  fact  be- 
tween these  cases  and  those  in  which  some  micrococci  are 
known  to  be  the  definite  cause. 

Pathology. — Acute  hydrocephalus  may  be  due  to  a 
number  of  different  causes.  First,  to  venous  stasis,  such  as 
occurs  in  connection  with  severe  heart  disease.  Secondly, 
venous  stasis  in  the  larger  veins  at  the  base,  due  to  neoplasm 
in  the  posterior  cranial  fossa,  may  also  be  the  direct  cause  of 
acute  dropsy  of  the  brain.  Thirdly,  stasis  in  the  lymphatic 
vessels,  most  frequently  due  to  tumors  in  the  posterior  fossa, 
causing  compression  of  the  subarachnoid  lymph  spaces  and 
of  the  aqueduct  of  Sylvius,  are  a  sufficient  cause  of  acute 
hydrocephalus.  Fourth,  any  acute  inflammatory  process 
of  the  brain  or  of  the  meninges  may  lead  to  this  form  of 
meningitis.     The  exact  symptomatology  of  an  affection  of 


HYDROCEPHALUS.  423 

this  nature  occurring  in  a  young  person  may  be  illustrated 
by  the  following  history  taken  from  Quincke  : 

A  boy,  nine  years  old,  who  was  said  always  to  have  had  a  large  cranium, 
was  struck  upon  the  back  of  the  head.  He  was  unconscious  for  a  few  min- 
utes, and  after  the  accident  complained  of  pains  in  the  occiput,  which  were 
generally  slight,  but  occasionally  much  increased.  With  increasing  headaches 
the  boy  grew  pale,  vomited,  showed  slight  rigidity  of  the  neck,  and  retraction 
of  the  head.  The  horizontal  circumference  of  the  head  six  months  after- the 
accident  was  54^  ctm.  No  pain  on  percussion  of  the  head,  but  pressure  upon 
the  upper  cervical  spinous  processes  was  painful.  Vision  much  diminished. 
The  right  eye  had  fair  perception  of  light ;  with  the  left  the  boy  was  able  to 
count  fingers.  The  pupils  moderately  dilated,  reacting  sluggishly;  both 
papilla;  were  atrophic.  All  movements  were  perfect.  Serous  fluid  was  with- 
drawn in  considerable  quantity  on  two  different  occasions  by  Quincke  through 
a  puncture  in  the  lumbar  region.  Upon  this  there  was  improvement  in  the 
headaches,  but  sight  remained  permanently  impaired. 

Chronic  Hydrocephalus. — From  this  category  of 
cases  we  may  at  once  exclude  those  in  which  the  increase  of 
intracranial  fluid  is  of  a  simple  compensatory  nature.  Such 
an  increase  is  found  in  many  instances  of  congenital  imper- 
fect development  of  the  brain.  A  skull  of  average  dimen- 
sions sometimes  harbors  a  very  small  brain,  the  cranial  cav- 
ity being  filled  by  an  increased  amount  of  fluid.  The  fluid 
causes  a  bulging  of  all  the  cranial  bones.  The  accumulation 
of  fluid  during  the  intra-uterine  period  may  increase  the  size 
of  the  head  to  such  an  extent  as  to  make  it  a  serious  obstacle 
to  normal  delivery. 

Deficient  brain  development  is  at  times  limited  to  one- 
half  of  the  brain.  An  entire  hemisphere  may  be  trans- 
formed into  a  large  serous  sac,  while  the  other  half  presents 
tolerably  normal  appearance.  An  entire  hemisphere  may 
consist  of  nothing  more  than  a  superficial  layer  of  gray 
matter  bordering  upon  a  huge  cyst  which  communicates 
with  the  ventricles.  I  have  had  opportunity  to  be  present 
at  an  operation  upon  such  a  brain  in  which  a  cyst  was  di- 
agnosticated, but  the  size  of  the  cyst  not  suspected.  On 
opening  this  cyst  the  surgeon's  probe  could  be  passed  many 
inches  forward  and  backward,  showing  that  the  greater 
part  of  the  hemisphere  consisted  of  this  cystic  mass.  The 
boy  died  about  two  weeks  after  the  operation,  when  it  was 


424  THE  NERVOUS  DISEASES   OF   CHILDREN. 

■found  that  the  entire  hemisphere  consisted  of  nothing  else 
than  a  small  layer  of  gray  matter  bordering  a  huge  cystic 
cavity.  But  all  these  forms,  however  interesting  they  may 
be,  have  comparatively  little  value,  and  are  far  less  frequent 
than  those  in  which  there  is  a  condition  of  congenital  hy- 
drocephalus. 

Congenital  Hydrocephalus. — For  reasons  which  are 
not  well  known  an  excessive  accumulation  of  fluid  may  occur 
within  the  ventricular  cavities  during  the  intra-uterine  pe- 
riod. The  condition  is  found  in  children  of  absolutely 
healthy  parents  after  normal  pregnancies,  and  in  families  in 
which  all  other  children  have  been  entirely  healthy.  In 
some  families,  however,  several  children  are  born  afflicted 
with  this  same  trouble  ;  one  or  the  other  may  survive  while 
the  majority  are  either  still-born  or  die  very  soon  after 
birth.  Syphilis  of  the  parents,  alcoholism,  tuberculosis  in 
the  father  or  mother,  emotional  excitement  of  the  mother 
during  pregnancy,  injuries  to  the  mother  during  this  same 
state,  all  these  factors  have  been  held  responsible  for  the 
condition,  and  no  doubt  are  operative  in  some  of  these 
patients,  while  in  the  majority  the  actual  cause  remains  un- 
known. If  hydrocephalus  is  present  at  the  time  of  birth 
the  amount  of  fluid  present  may  go  on  increasing  within 
the  first  few  days,  or  as  long  as  the  child  lives. 

In -this  connection  the  observations  made  upon  an  infant  whom  I  had 
occasion  to  examine  in  1893  may  be  of  some  interest.  When  I  saw  it 
for  the  first  time  it  was  four  weeks  old.  The  mother  had  given  birth  to  one 
other  girl,  three  years  previously,  that  had  remained  healthy  and  showed  no 
signs  of  internal  hydrocephalus.  The  father  was  a  man  of  remarkably  vig- 
orous health.  During  this  second  pregnancy  the  mother  menstruated  regu- 
larly. On  account  of  the  size  of  the  head  labor  was  extremely  difficult,  but 
the  child  was  uninjured  when  born.  The  measurements  which  were  taken 
at  three  different  occasions  will  show  the  rapid  increase  of  the  fluid,  as  indi- 
cated by  the  rapid  increase  in  the  head  measurements  : 

Aug.  6.        Aug.  8.       Aug.  10. 
Inches.         Inches.        Inches. 

Horizontal  circumference 20^  21%  22 

Naso-occipital  measurement 13^2  I2^  H/^ 

Bin-auricular  measurement \2}i  13K  13 

On  the  9th,  between  the  second  and  third  measurements,  a  large  amount 
of  fluid  was  withdrawn  by  tapping  the  fontanelles,  but  it  will  be  noticed  that 


HYDROCEPHALUS.  425 

the  fluid  was  rapidly  replaced.  The  child  lingered  on  for  another  week  and 
then  died,  after  having  become  greatly  emaciated.  More  than  a  week  before 
its  death  it  could  not  be  fed  by  the  mouth  or  per  rectum. 

But  all  cases  of  congenital  hydrocephalus  do  not  take 
such  a  serious  turn,  and  in  some  of  them  the  accumulation 
of  fluid  ceases  and  a  tolerably  fair  and  even  normal  mental 
development  may  ensue.  The  protruding  occipital  bones 
clearly  visible  on  so  many  bald  heads  point  to  a  moderate 
amount  of  internal  hydrocephalus  in  the  earlier  years  of 
life.  Some  of  these  may  have  been  acquired  in  the  earlier 
period  of  life  and  are  not  necessarily  congenital  in  origin. 
The  fluid  accumulates  most  readily  within  the  lateral  ven- 
tricles, both  in  the  anterior  and  in  the  posterior  horns.  The 
aqueduct  of  Sylvius  may  be  distended  into  a  funnel  shape 
by  the  increased  fluid,  but  the  fourth  ventricle,  as  a  rule, 
suffers  very  little.  The  brain  is  compressed  by  the  ac- 
cumulation of  fluid,  and  is  often  so  flattened  and  thinned 
that  its  thickness  is  not  greater  than  that  of  ordinary  thick 
paper.  The  white  matter  seems  to  yield  more  readily  than 
the  gray,  and  I  have  been  struck,  in  a  number  of  different 
brains,  by  the  fact  that  the  function  of  the  parts  remained 
normal  in  spite  of  the  extreme  thinness  of  the  cerebral  tissue. 
Thus  in  one  instance  in  which  both  occipital  lobes  were  re- 
duced to  the  thickness  of  paper,  sight  had  remained  entirely 
normal  up  to  the  age  of  two  years,  at  which  time  the  patient 
died.  The  membranes  are,  as  a  rule,  normal,  or  else  show 
only  very  slight  thinning,  and  on  microscopical  examination 
only  the  slightest  traces  of  acute  inflammation  or  none  at 
all.  The  choroid  plexuses  are  generally  thickened,  as  is 
also  the  limiting  membrane  of  the  ventricles. 

The  fontanelles  bulge  and  pulsate  distinctly.  The  con- 
figuration of  the  skull  is  modified  by  the  excess  of  internal 
hydrocephalic  fluid.  If  the  hydrocephalus  is  congenital,  or 
if  it  has  occurred  in  very  early  life,  the  bones  yield  to  the 
increased  pressure  within  and  the  skull  becomes  distended 
in  all  directions.  In  keeping  with  the  greater  accumulation 
of  fluid  in  the  anterior  and  posterior  horns  of  the  ventricles 
and  the  protrusion  of  corresponding  parts  of  the  brain,  the 
greatest  amount  of  bulging  is  observed  in  the  frontal  and  oc- 
cipital bones  respectively.     The  effect  of  the  excessive  ac- 


426  THE  NERVOUS  DISEASES   OF   CHILDREN. 

cumulation  of  fluid  in  the  posterior  horns  of  the  ventricles 
results  in  a  great  bulging  of  the  occipital  bones,  with  marked 
increase  in  the  transverse  occipital  diameter  of  the  skull. 
If  the  child  survives,  the  congenital  internal  hydrocephalus 
gives  rise  to  a  number  of  symptoms  pointing  to  deficient 
cerebral  development.  On  account  of  the  size  and  weight 
of  the  head  the  child  is  rarely  able  to  carry  the  head  unsup- 
ported. As  it  grows  older  it  may  be  ahle  to  sit  in  a  chair, 
but  the  head  generally  inclines  to  one  side  or  the  other,  or 
forward,  in  which  case  it  may  rest  upon  the  chest.  Defec- 
tive mental  development  is  present  in  the  majority  of  these 
cases,  ranging  between  total  idiocy  and  varying  degrees  of 
imbecility  ;  some  of  the  children  acquiring  a  slight  use  of 
language  and  others  learning  to  utter  only  a  few  words. 
Wyss  states  that  of  forty-five  hydrocephalic  children  only 
five  were  able  to  attend  school.  Cerebral  palsies  with  typi- 
cal spastic  contractures  are  quite  frequent,  and  convulsive 
seizures  amounting  to  a  chronic  epilepsy  often  add  to  the 
burdens  of  the  child,  and  to  the  distress  of  the  mother  who 
is  to  care  for  it.  In  some  cases  there  is  total  blindness,  due 
to  optic-nerve  compression,  but  the  most  surprising  feature 
of  all  is  the  remarkable  preservation  of  the  brain  functions. 

Acquired  Internal  Hydrocephalus. — Chronic  internal  hydro- 
cephalus, not  of  congenital  origin,  is  a  rare  disease,  and  it  is  at  least  in  rare 
instances  only  that  the  diagnosis  of  such  a  form  of  internal  hydrocephalus 
can  be  made,  and  if  made,  the  cause  which  gives  rise  to  it  is  so  far  more  im- 
portant that  the  internal  hydrocephalus  is  a  mere  incident  and  does  not  de- 
serve much  notice.  This  acquired  internal  hydrocephalus  may  be  of  pri- 
mary origin  or  it  may  be  due  to  some  mechanical  obstruction  causing  venous 
stasis.  The  primary  form  may  be  a  symptom  of  severe  general  anaemia,  and 
under  such  conditions  it  is  a  counterpart  of  serous  transudation  occurring  in 
various  cavities  of  the  body.  It  may  also  occur  in  children  who  have  rick- 
ets, or  in  those  whose  skull  is  sufficiently  yielding  to  permit  enlargement  of 
the  cranial  cavity.  The  increase  of  fluid  is  to  a  certain  extent  compensatory 
in  such  cases. 

Internal  hydrocephalus  due  to  mechanical  obstruction  of  the  veins  of 
Galen,  was  first  described  by  Whytt.  The  commonest  form  of  obstruction 
is  that  due  to  tumor  in  the  posterior  fossa.  Through  such  obstructions  the 
foramen  of  Majendie  may  become  occluded  and  dilatation  of  the  third  ventricle 
will  result ;  in  the  case  of  occlusion  of  the  foramen  of  Monro,  a  distention 
will  take  place  in  the  lateral  ventricle,  but  increased  secretion  of  serous 
fluid  may  be  sufficient  to  cause  distention  of  all  the  ventricles  without  any 


HYDROCEPHALUS.  427 

obstruction  whatever.  On  the  other  hand,  a  meningitis  may  be  sufficient  to 
occlude  these  small  openings  connecting  the  ventricles  with  one  another, 
and  may  thus  become  the  actual  cause  of  an  internal  hydrocephalus.  This 
special  cause  of  internal  hydrocephalus  is  of  interest  only  in  those  compara- 
tively few  cases  which  survive  a  severe  attack  of  meningitis.  A  slight  in- 
crease of  intracranial  fluid  is  present  in  almost  every  case  of  intracranial 
growth,  and  is  responsible  for  many  of  the  general  cerebral  symptoms  ac- 
companying such  diseases ;  but  as  we  said  above,  the  recognition  of  tumor 
is  so  much  more  important  than  the  diagnosis  of  an  accompanying  hydro- 
cephalus that  the  latter  loses  very  much  in  importance. 

The  symptomatology  of  the  acquired  form  of  internal  hydrocephalus  dif- 
fers in  no  respect  from  that  of  other  chronic  forms  except  that  the  symptoms 
develop  with  some  suddenness  ;  vertigo,  dizziness,  stupor,  coma,  convulsions, 
contractures,  and  amblyopia,  preceded  possibly  by  a  condition  of  hemianopsia, 
are  the  symptoms  which  should  be  looked  for  in  these  patients.  It  stands  to 
reason  that  if  the  internal  hydrocephalus  originates  at  the  time  when  the  bones 
are  still  yielding,  the  injury  to  the  brain  and  the  symptoms  resulting  therefrom 
will  be  far  less  marked  than  they  will  be  if  it  occurs  after  complete  ossification 
of  all  the  sutures  and  bones.  After  the  sutures  have  been  closed  the  increas- 
ing hydrocephalus  may  force  them  open  again.  I  have  not  observed  this  in 
any  of  the  primary  forms  of  hydrocephalus,  but  have  seen  it  occur  in  a  child 
of  four  years,  who  died  of  cerebellar  tumor  with  enormous  internal  and  ex- 
ternal hydrocephalus.  The  acquired  internal  hydrocephalus  runs  very  much 
the  same  course  as  the  chronic  form,  and  is  very  apt  to  terminate  fatally  in  a 
few  weeks  or  months.  Moderate  cases  may  survive  for  a  longer  period  of  time. 

The  diagnosis  of  all  forms  of  internal  hydrocephalus  de- 
pends entirely  upon  the  association  of  an  enlargement  of  the 
cranial  cavity  with  symptoms  which  point  to  an  increase  of 
intracranial  pressure.  The  exact  form  of  hydrocephalus, 
whether  it  be  congenital  or  acquired,  can  be  diagnosticated 
only  with  reference  to  the  history  of  the  individual.  In  en- 
deavoring to  determine  whether  the  hydrocephalus  is  in- 
ternal or  external,  we  should  remember  that  the  external 
hydrocephalus  is  more  apt  to  be  congenital  and  to  be  asso- 
ciated with  symptoms  of  defective  mental  development ;  and 
furthermore,  that  bulging  of  the  frontal  or  occipital  bones 
only  accompanies  internal  hydrocephalus ;  whereas,  a  gen- 
eral enlargement  of  the  skull  in  every  diameter,  with  bulg- 
ing fontanelles  and  sutures  forced  apart,  indicates  the  pres- 
ence of  external  hydrocephalus,  which  is  in  most  cases  as- 
sociated with  distention  of  the  ventricles  as  well.  But  in 
many  children  the  distinction  between  external  and  internal 
hydrocephalus  is  difficult  to  make,  for  the  internal  hydro- 


428  THE   NERVOUS  DISEASES   OF   CHILDREN. 

cephalus  may  have  so  thinned  out  the  cerebral  substance  as 
to  bring  the  ventricular  fluid  very  near  the  surface. 

Further  difficulties  of  differential  diagnosis  which  arise 
occasionally  are  to  distinguish  from  hydrocephalus  the  en- 
largement of  the  skull  due  to  rickets,  to  syphilis,  or  to  thin- 
ning of  the  skull  resulting  from  other  causes.  In  the  case  of 
rickets,  the  entire  history  of  the  disease  will  give  a  clew,  and 
other  symptoms  of  rickets  will  be  present  and  will  indicate 
the  true  nature  of  the  enlargement  of  the  head.  But  both 
rickets  and  hydrocephalus  are  not  infrequently  associated 
with  each  other.  An  increase  in  the  size  of  the  skull  may 
simulate  the  condition  of  hydrocephalus.  Such  thickening 
is  extremely  rare,  but  is  met  with  in  the  subjects  of  hered- 
itary syphilis.  I  have  seen  this  but  once,  in  a  boy,  aged  six 
years,  in  whom  there  were  other  signs  of  hereditary  syph- 
ilis ;  yet  the  skull  is  never  so  much  enlarged  as  in  the  ex- 
treme cases  of  hydrocephalus,  and  generally  impresses  one 
as  being  unusually  hard. 

PROGNOSIS. — The  prognosis  of  hydrocephalus  is  always 
serious,  but  depends  greatly  upon  the  original  cause.  Fort- 
unately a  goodly  number  of  children  with  varying  degrees 
of  hydrocephalus  not  only  live  a  number  of  years,  but  get 
well.  The  congenital  cases  are  more  rapidly  fatal  than  the 
acquired  form,  and  the  prospects  of  a  normal  mental  de- 
velopment are  always  slight,  though  remarkable  exceptions 
may  occur.  Measurements  of  the  skull  taken  at  regular 
intervals  will  enable  the  physician  to  gauge  the  rate  of  in- 
crease in  the  accumulation  of  fluid,  and  will  help  him  to 
prognosticate  the  future  course  of  the  disease. 

Treatment. — In  the  milder  forms  of  hydrocephalus,  the 
iodides  and  the  preparations  of  iron  will  do  good  service. 
In  the  severer  forms  of  hydrocephalus  the  attempt  should  be 
made  to  absorb  some  of  the  fluid  which  is  present  in  excess 
by  the  use  of  the  mercurials  and  diuretic  measures.  I  can- 
not state  that  I  have  seen  any  favorable  results  following 
upon  this  course  of  treatment,  though  some  temporary  re- 
lief has  been  afforded  in  a  few  cases.  The  treatment  is, 
however,  a  thoroughly  rational  one.  In  almost  every  form 
of  acute  and  increasing  hydrocephalus,  however  little  faith 
he  may  have  in  the  efficacy  of  the  method,  the  physician  or 


HYDROCEPHALUS.  429 

surgeon  will  be  tempted  to  follow  the  example  of  Keen, 
Kocher,  Bergmann,  Chipault,  to  tap  the  ventricles,  and  thus 
drain  away  some  of  the  fluid.  The  fluid  is  replenished  so 
rapidly  that  very  little  good  can  be  effected  in  this  way,  and 
if  it  is  drained  off  suddenly  the  danger  of  collapse  is  very 
great.  Henschen's  statistics  have  been  most  discouraging. 
At  the  present  day,  lumbar  puncture  will  take  the  place  of 
this  procedure,  but  is,  of  course,  useless  in  those  cases  in 
which  the  communication  between  the  ventricles  and  the 
cerebro-spinal  canal  is  interrupted.  Instead  of  draining 
away  a  large  quantity  at  one  time,  it  seems  much  more 
rational  to  attempt  some  method  of  gradual  drainage. 

Every  other  treatment,  including  the  injections  of  iodine,  the  application 
of  elastic  bands  about  the  head,  and  of  compression  of  various  kinds,  is  use- 
less. I  have  often  attempted  to  reduce  the  skull  by  applying  bandages  more 
or  less  firmly  around  the  head,  but  the  result  has  in  every  instance  been  equally 
unsatisfactory.  If  the  skull  is  firmly  compressed  the  internal  pressure  is  nat- 
urally increased,  and  if  the  compression  is  not  firm  enough  the  bandages  will 
do  no  good. 


CHAPTER   XXV. 

INFANTILE   CEREBRAL   PALSIES. 
(SPASTIC   HEMIPLEGIA,   DIPLEGIA,    PARAPLEGIA.) 

The  investigators  of  recent  years  have  with  few  ex- 
ceptions endorsed  the  classification  of  the  cerebral  palsies, 
as  given  in  the  first  edition  of  this  book.  We  may  divide 
these  palsies  into  three  groups  according  to  time  of  onset — 
during  the  intra-uterine  period,  during  labor,  or  after  birth. 
A  clearer  understanding  of  all  these  disorders  will  be 
reached  by  discussing  them  together,  comparing  and  con- 
trasting the  chief  features  of  the  one  series  with  those  of  the 
other  groups. 

The  subject  is  not  a  new  one,  yet  the  various  forms  of  cerebral  palsies  did 
not  attract  the  attention  which  they  deserved  until  two  decades  ago.  A  clear 
account  of  their  clinical  symptoms  was  given  by  Von  Heine  in  the  second 
edition  of  his  book  on  "  Infantile  Spinal  Palsies  "  (i860).  Heine  distin- 
guished between  the  typical  cases  of  infantile  spinal  paralysis  and  those  of  a 
purely  cerebral  character.  Before  his  day  some  of  the  older  French  authors 
— Cazauvielh,  Breshet,  Cruveilhier — had  studied  the  atrophic  changes  in  the 
brains  of  children  who  had  been  afflicted  with  various  forms  of  palsy.  These 
writers  were  interested  in  the  anomalies  of  brain  structure  rather  than 
in  the  clinical  features  presented  by  such  cases.  In  1842  Henoch  published 
a  dissertation  entitled  "  De  Atrophia  Cerebri,"  in  which  he  described  the 
brain  condition  associated  with  infantile  cerebral  hemiplegia.  The  chief  ad- 
vances were  due,  however,  to  Cotard,  a  pupil  of  Charcot,  who  in  1868  pub- 
lished "  A  Study  on  the  Partial  Atrophy  of  the  Brain,"  in  which  he  analyzed 
the  different  morbid  processess  to  which  such  atrophy  might  be  due.  Cotard 
was  first  also  to  recognize  the  importance  of  traumatic  encephalitis  as  a  factor 
in  the  causation  of  these  conditions.  A  special  study  of  brain  defects  was 
made  by  Heschl,  who  introduced  the  term  "  porencephaly,"  and  by  Kundrat, 
who  in  1882  published  a  monograph  on  this  same  subject,  in  which  he  dis- 
tinguished between  congenital  and  acquired  porencephaly,  and  attempted  to 
explain  all  these  conditions  by  attributing  them  to  a  form  of  anaemic  necrosis. 
In  1883  Audry  published  a  collection  of  one  hundred  and  three  cases  of  this. 


INFANTILE    CEREBRAL   PALSIES.  43 1 

peculiar  condition.  Lobar  sclerosis,  a  condition  to  which  Cotard  had  called 
attention,  was  made  the  special  subject  of  papers  written  by  Bourneville, 
Richardiere,  Jendrassik,  and  Marie.  The  last  author  introduced  a  decided 
advance  in  the  discussion  of  the  subject  by  connecting  this  lobar  sclerosis 
with  changes  in  the  blood-vessels,  and  still  the  character  of  the  initial  affec- 
tion remained  rather  doubtful.  Quite  apart  from  all  these  French  and 
German  authors,  Little,  in  1853,  had  studied  carefully  the  spastic  palsies  and 
rigidities  of  children,  and  showed  that  they  were  largely  due  to  protracted 
labor  and  premature  delivery.  The  conditions  due  to  these  two  causes  have 
since  been  known,  particularly  among  German  and  French  authors,  as  "  Little's 
Disease."     (Cf.  page        .) 

A  fresh  impetus  was  given  to  the  study  of  all  these  palsies  by  the  lecture 
of  Strtimpell,  delivered  in  1884,  who  declared  that  acute  infantile  spastic 
hemiplegia  was  due  to  a  primary  acute  encephalitis  affecting  the  gray  matter 
of  the  cord.  He  insisted  upon  an  analogy  between  this  acute  cerebral  and 
the  acute  spinal  palsies,  and  in  order  to  fortify  the  resemblance  between  the 
two  sets  of  disease,  proposed  the  term  "  polio  encephalitis  "  as  the  cerebral 
counterpart  of  "  polio-myelitis."  The  theory  advanced  by  Strumpell  was  in- 
genious ;  there  is  more  clinical  than  anatomical  evidence  to  support  it.  We 
can  not,  even  at  this  day,  agree  with  Leube,  who  states  that  "  hemiplegia  in 
young  children  should  in  the  first  instance  be  considered  to  be  the  result  of 
a  former  acute  encephalitis."  The  truth  is  that  encephalitis  is  the  rarest 
cause  of  these  palsies,  and  should  not  be  considered  at  all,  unless  every  other 
morbid  state  can  be  safely  excluded.     But  of  this  later  on. 

StrumpeH's  lecture  was  the  starting-point  of  the  innumerable  studies 
which  have  led  to  the  clearer  recognition  of  the  true  pathology  of  these 
disorders.* 

The  brain  palsies  of  children  are  far  more  frequent  than 
they  are  generally  supposed  to  be.  In  the  Hospital  for 
Ruptured  and  Crippled  in  this  city,  Dr.  Townsend,  at  my 
suggestion,  was  kind  enough  to  tabulate  the  cases  of  spinal 
and  cerebral  infantile  palsies  which  presented  themselves 
for  treatment  at  that  institution  for  a  definite  period  of  time. 
His  statistics  show  that  during  that  period  ninety-one 
patients  with  infantile  cerebral  palsies,  and  one  hundred  and 
forty-two  cases  of  infantile  spinal  palsies  were  received  for 
treatment.  I  had,  during  a  period  of  five  years,  seen  over 
three  hundred  cerebral  spastic  palsies,  but  this  may  be 
due  to  the  fact  that  many  physicians  knowing  my  interest 

*  Among  the  many  writings  the  most  prominent  are  those  of  Bernhardt,  Wallenberg, 
Kast,  Moebius,  Feer,  Freud  and  Rie,  and  Oppenheim,  in  Germany  ;  of  Marie,  Cotard, 
Audry,  Gibbotteau,  in  France ;  of  Ross,  Hadden,  Gowers,  and  Ashby,  in  England  ; 
in  this  country  numerous  articles  have  been  contributed  to  this  subject  by  Weir 
Mitchell,  Sarah  McNutt,  Lovett,  Osier,  Peterson,  Sachs,  Starr,  Spiller,  and  Dercum. 


432 


THE  NERVOUS  DISEASES   OF  CHILDREN. 


in  the  subject  were  at  special  pains  to  refer  such  cases  to 
me. 

Whatever  the  time  or  manner  of  origin  may  have  been, 
all  cases  of  cerebral  palsy  in  children  are  so  similar  in  many 
respects  that  we  may  attempt  to  study  the  symptoms  com- 
mon to  all.  These  various  forms  of  cerebral  palsies  are 
observed  most  frequently  in  the 
earlier  years  of  life,  from  the  time 
of  birth  up  to  the  age  of  ten  years, 
and  even  later,  but  by  far  the  great- 
er majority  of  them  occur  during 
the  first  three  years  of  life.  The 
paralysis  is  of  the  distinctly  spas- 
tic order  and  according  to  the  dis- 
tribution of  the  palsy  we  may  dis- 
tinguish between  a  hemiplegia  and 
diplegia  (double  hemiplegia)  or 
paraplegia.  Monoplegias  are  rela- 
tively rare.  The  rigidity  of  the 
muscles,  the  contractures  resulting 
from  the  same  causes,  and  increase 
of  all  the  deep  reflexes  are  the  con- 
stant accompaniment  of  these  pal- 
sies. Coma  and  convulsions  occur 
in  the  initial  stage  of  the  acute 
forms,  and  the  convulsions  at  least 
are  often  repeated  during  the  later 
stages  of  the  disease.  The  cere- 
bral palsies  of  children  are  more 
commonly  associated  with  coma 
and  convulsions  than  are  those  of 
the  adult ;  the  former  are  generally 
due  to  cortical  processes,  the  latter 
to  intra-cerebral  conditions.  The  frequent  repetition  of 
convulsions  is  equivalent  to  the  establishment  of  an  epi- 
lepsy which  may  continue  throughout  life,  and  the  same 
changes  which  have  caused  the  epilepsy  may  also  be  re- 
sponsible for  the  defective  mental  development,  which  may 
range  from  weak-mindedness  to  marked  imbecility  and  com- 
plete idiocy.      Disturbances  of  motion,  associated,   ataxic 


Fig.  105.  —  Left  Hemiplegia. 
Onset  in  Second  Year.  Con- 
tractures of  arm  and  fingers. 
(See  also  Fig.  75.) 


INFANTILE    CEREBRAL   PALSIES.  433 

athetoid,  choreiform,  and  even  cataleptic  movements,  occur 
quite  often  in  connection  with  these  diseases.  All  of  them 
may  be  grouped  under  the  general  heading  of  post-para- 
lytic (not  necessarily  post-hemiplegic)  disturbances.  Apha- 
sia, so  common  in  apoplectic  disorders  of  the  adult,  is  a 
rarer  complication  in  infantile  palsies. 

Among  the  negative  symptoms  which  are  of  the  greatest 
importance  in  attempting  a  differential  diagnosis  between 
the  cerebral  spastic  and  the  spinal  palsies,  we  may  mention 
the  entire  absence  of  changes  in  the  electrical  reactions  and 
the  development  of  only  slight  atrophy  in  association  with 
the  palsy.  Disturbances  of  sensation  are  rare,  but  as  these 
are  absent  in  the  spinal  forms  as  well,  they  help  little  in 
attempting  a  differential  diagnosis  between  these  two  im- 
portant series  of  diseases. 

Let  us  now  take  up  the  symptoms  seriatim,  and  in  doing 
so  we  shall  be  able  to  develop  the  many  interesting  features 
of  infantile  cerebral  palsies. 

Distribution  of  Paralysis.— The  following  analysis  of  225  cases  is 
based  upon  the  collections  made  by  Peterson  and  myself  : 

Males.  Females.  Total. 

Right  hemiplegia. . . 51  30  81 

Left  hemiplegia 40  35  75 

Diplegia 21  18  39 

Paraplegia : 22  8  30 

Total 134  91  225 

Monoplegias  are  so  rare  that  they  scarcely  enter  into  the  consideration  of 
the  problem.  From  the  above  statistics  it  will  be  inferred  that  right  and  left 
hemiplegias  are  more  frequent  that  the  bilateral  forms  of  palsy,  which  is 
equivalent  to  saying  that  unilateral  cerebral  processes  are  more  common  than 
double  cerebral  lesions.  There  is  no  difference  more  striking  between  the 
adult  and  the  infantile  palsies  than  that  implied  in  the  relatively  large  per- 
centage of  cases  of  cerebral  diplegia  and  paraplegia.  In  the  adult  a  bilateral 
cerebral  lesion  is  a  great  rarity,  but  in  children  diplegias  constitute  about 
fifteen  per  cent,  of  all  the  cases,  and  paraplegia  about  fourteen  per  cent. 
This  difference  between  the  adult  and  infantile  brain  is  due  first  of  all  to  the 
fact  that  both  hemispheres  in  children  are  exposed  to  the  same  external  in- 
juries at  birth,  and  even  if  the  disease  is  acquired  later  in  life  the  two  hemi- 
spheres seem  often  to  be  affected  simultaneously,  and  much  more  frequently 
than  in  later  years. 


434 


THE   NERVOUS  DISEASES   OF  CHILDREN. 


In  my  own  statistics  boys  were  affected  a  little  more  often  than  girls,  but 
the  difference  is  hardly  great  enough  to  be  made  much  of,  and  Gowers  has 
found  just  the  reverse  to  be  true.  Osier  found  that  girls  and  boys  were 
affected  in  about  equal  proportion. 

The  onset  of  a  cerebral  palsy  will  vary  naturally  according  to  the  three 
groups  which  we  have  established.  It  would  seem  entirely  superfluous  to 
make  any  special  remarks  regarding  the  time  at  which  the  prenatal  palsies 
begin.  It  might  be  sufficient  to  say  that  they  begin  during  the  intra-uterine 
period,  and  that  such  children  are 
born  palsied.  It  is  a  matter  of  fact 
that  the  symptoms  of  such  intra- 
uterine cerebral  defects  are  not  al- 
ways manifest  at  birth,  and  indeed  a 
number  of  months  may  pass  before 
it  becomes  evident  to  the  physician 
that  the  child's  cerebral  condition  is 
not  a  normal  one.  A  very  fair  per- 
centage of  cases  which  appear  to 
begin  during  the  first  years  of  life 
could  properly  enough  be  classified 
among  the  prenatal  palsies,  and  if 
in  any  child  the  first  symptoms  of 
a  cerebral  palsy  are  noticed  sev- 
eral months  after  birth,  and  yet  the 
period  of  labor  was  entirely  normal, 
I  should  be  inclined  to  classify  that 
case  rather  among  the  prenatal  pal- 
sies than  among  the  birth  palsies. 

The  birth  palsies  begin  naturally 
enough  with  the  period  of  labor. 
The  history  in  these  cases  clearly 
shows  that  the  labor  was  either  ex- 
cessively prolonged,  or  that  an  in- 
strumental delivery  was  resorted  to, 
in  which  case  the  brain  has  evident- 
ly suffered  mechanical  injury.  Pre- 
mature delivery  is  responsible  for 
many  cerebral  palsies ;  but  the  symptoms  may  not  be  fully  developed  until 
months  after  birth. 

The  age  of  onset  in  the  acute  or  acquired  forms  varies  considerably,  and 
yet  the  chief  statistics  agree  in  this  that  fully  two-thirds  of  all  the  cases  be- 
gin in  the  first  three  years  of  life.  Some  maintain  that  by  far  the  largest 
proportion  begin  during  the  first  year,  but  I  am  not  inclined  to  accept  this 
view,  since  such  a  list  would  include  a  large  number  of  cases  which  are  more 
properly  congenital,  although  the  symptoms  do  not  become  manifest  until 
some  time  after  birth.  Among  a  total  of  one  hundred  and  forty  cases,  Pe- 
terson and  I  found  that  but  five  began  in  the  eighth  year,  and  four  cases 


Fig.  106.  —  Right  Hemiplegia,  with  Con- 
tractures and  Retarded  Growth  of  Arm. 
Onset  of  disease  at  eight  years  of  age, 
following  typho-malarial  fever. 


INFANTILE    CEREBRAL  PALSIES.  435 

between  the  ages  of  fourteen  and  fifteen  years,  but  these  were  cases  of  hemi- 
plegia, and  it  is  of  some  interest  to  note  that  no  patients  with  diplegia  or 
paraplegia  were  observed  after  the  third  year,  showing  that  the  causes  which 
lead  to  double  cerebral  lesions,  and  possibly  the  condition  which  permits 
double  lesions,  cease  with  the  very  earliest  years  of  life.  The  majority  of 
diplegias  and  paraplegias  are  either  congenital  or  occur  during  the  first  year, 
which  can  be  easily  explained  by  the  fact  that  the  injuries  sustained  during 
labor  are  largely  responsible  for  these  special  types. 


Etiology. — The  true  etiology  of  the  prenatal  cerebral 
palsies  is  often  rather  mysterious.  We  have  to  resort  to 
vague  statements  that  they  are  due  to  hereditary  taints,  and 
as  a  matter  of  fact  such  palsies  occur  frequently  enough 
in  families  with  a  decided  history  of  neurotic  taints  of 
one  kind  or  another,  with  a  history  of  hereditary  epilepsy 
or  of  hereditary  insanity.  I  have  a  number  of  times  ob- 
served such  children,  in  whom  an  hereditary  taint  was  pres- 
ent in  the  families  of  both  father  and  mother.  The  next 
most  important  factor  is  unquestionably  the  occurrence  of 
some  accident  or  injury  to  the  mother  during  the  period  of 
pregnancy.  This  was  to  be  traced  distinctly  in  a  number 
of  cases  of  my  own.  The  manner  in  which  such  trauma- 
tism may  do  actual  injury  to  the  brain  of  the  child,  was 
clearly  shown  by  a  case  which  was  cited  by  Cotard.  The 
mother  of  the  child  sustained  a  blow  against  the  abdomen 
during  pregnancy.  The  child,  which  was  still-born  three 
months  after  the  injury,  exhibited  extreme  contractures  of 
both  left  upper  and  lower  extremities.  On  examination  of 
the  brain  an  old  lesion  was  discovered  in  the  right  hemi- 
sphere in  the  vicinity  of  the  lateral  ventricle. 

Illness  of  the  mother  during  pregnancy,  exhausting 
fevers,  particularly  such  as  accompany  pneumonia  and 
typhoid,  ursemic  convulsions,  and  severe  fright,  are  other 
causes  which  have  been  made  out  in  cases  of  prenatal 
palsies.* 

*  Osier  reports  (in  Teratologia,  vol.  ii.,  No.  1),  upon  the  case  of  a  young  woman 
who  died  of  typhoid  fever.  "The  uterus  contained  a  foetus,  apparently  about  six 
months  old.  .  .  .  The  brain  was  very  soft ;  the  right  hemisphere  was  normal,  but 
in  the  left  .  .  .  was  a  cavity  with  ragged,  irregular  walls,  containing  a  large,  recent 
clot,  which  had  broken  through  the  ganglia  into  the  lateral  ventricle  of  the  same  side. 
.     .     .     No  special  changes  were  noted  in  the  arteries." 


436  THE  NERVOUS  DISEASES   OF   CHILDREN. 

Hereditary  syphilis  does  not  play  an  important  part  in 
the  etiology  of  prenatal  palsies.  Erlenmeyer,  Osier,  Oppen- 
heim,  Koenig,  and  others  mention  it.  As  a  matter  of  fact 
I  have  been  able  to  convince  myself  of  the  influence  of 
syphilis  in  but  a  single  one  of  the  many  cases  which  I  have 
seen.  The  fact  that  syphilis  of  the  parents  so  frequently 
leads  to  still-births  may  account  in  a  measure  for  the  small 
part  played  by  this  special  affection  in  these  diseases. 

The  etiology  of  birth  palsies  is  a  very  simple  one.  It  is 
surprising  to  note  how  much  pressure  the  brain  and  skull 
will  tolerate  without  injury,  but  it  is  natural  that  harm 
should  occasionally  be  done.  Asphyxia  at  birth  has  since 
Little's  day  been  considered  a  most  potent  factor.  My 
own  studies  in  this  matter,  which  were  based  upon  a  very 
careful  collection  of  statistics,  have  proved  that  tedious 
labor  is  a  more  frequent  and  a  more  disastrous  factor  than 
instrumental  delivery.  Moreover,  these  birth  palsies  occur 
most  frequently  in  first-born  children. 

The  prolonged  compression  of  the  skull  during  the  last  months  of  preg- 
nancy would  seem  to  have  exercised  considerable  influence  in  this  direction, 
but,  of  course,  the  chief  damage  done  is  done  during  the  period  of  actual 
labor.  If  physicians  were  more  confident  of  the  safety  of  the  forceps  and  of 
their  own  skill  to  apply  the  same,  protracted  labor  would  not  be  as  powerful 
an  etiological  factor  as  it  is  at  the  present  time.  There  is,  therefore,  a  distinct 
inference  to  be  drawn  from  these  facts,  and  a  word  of  warning  should  be  ut- 
tered to  the  obstetrician  that,  other  things  being  equal,  and,  above  all,  the  life 
of  the  mother  not  being  in  danger,  it  is  wise  to  curtail  the  period  of  labor  as 
much  as  possible,  and  not  necessarily  to  wait  until  the  child's  heart  action  be- 
comes feeble.  Many  children  might  have  escaped  idiocy  and  epilepsy  if  the 
period  of  labor  had  been  properly  managed. 

Peterson  is  of  the  opinion  that  some,  though  a  very  small  percentage  (of 
birth  palsies)  may  be  due  to  cord  lesions  at  birth.  The  same  view  has  more 
recently  been  advocated  by  Dejerine.  H.  R.  Spencer,  among  one  hundred  and 
thirty  still-born  children,  found  hemorrhage  into  the  spinal  canal  and  cord 
in  thirty.  Little  suspected  that  "  spinal  meningitic  and  myelitic  affections 
may  play  a  considerable  part  in  the  phenomena  of  spastic  rigidity  "  ;  he  also 
refers  to  a  case  of  spastic  rigidity,  reported  by  Dr.  Marion  Sims,  in  which  a 
coagulum  of  blood  was  found  occupying  the  whole  length  of  the  spine. 

Acute  cerebral  palsies  may  be  due  to  a  variety  of  causes. 
The  acute  infectious  diseases  play  a  very  important  role  ; 
measles,  scarlatina,  typhoid,  small-pox,  and  even  a  tonsillitis 


INFANTILE    CEREBRAL   PALSIES.  437 

have  been  the  precursors  of  such  palsies.  The  same  have 
also  occurred  after  pneumonia  and  whooping-cough,  but  in 
the  case  of  the  latter  it  is  questionable  whether  the  palsy  is 
not  as  often  due  to  mechanical  injury  during  a  spasm  of 
coughing  as  to  the  effect  of  the  toxic  agent.  Fright— which 
Freud  has  interpreted  to  be  equivalent  to  a  psychic  trauma 
— is  an  occasional  cause  of  acute  cerebral  paralysis,  but 
actual  traumatic  violence  to  the  skull  is  a  much  more  pow- 
erful factor.  These  palsies  also  occur  after  simple  or  cere- 
brospinal meningitis,  after  an  exhausting  gastro-enteritis, 
and  after  other  severe  fevers  ;  but,  of  course,  there  is  always 
the  danger  of  making  a.  post  hoc  a  propter  hoc.  Among  ninety- 
one  cases  of  acquired  cerebral  palsy  the  exact  cause  could 
not  be  ascertained  in  twenty-seven. 

There  has  been  a  tendency,  particularly  among  French 
authors,  to  claim  that  all  cases  of  acquired  cerebral  palsy 
were  due  to  acute  infectious  diseases.  This  seems  to  me 
to  be  straining  the  point  altogether  too  much,  nor  is  there 
much  evidence  that  acute  cerebral  palsy  in  itself  represents 
an  acute  infectious  disease,  as  Strumpell  claimed  some  years 
ago.  As  a  matter  of  fact  only  relatively  few  of  the  cases 
of  acquired  cerebral  palsy  begin  with  fever,  coma,  and 
convulsions,  the  very  symptoms  which  Strumpell  thought 
most  characteristic  of  polio-encephalitis.  The  writer  has 
departed  from  his  former  views  to  the  extent  of  conced- 
ing that  a  small  number  of  the  acute  cerebral  palsies  of 
children  may  be  due  to  an  hemorrhagic  (infectious?)  en- 
cephalitis. 

The  importance  of  convulsions  as  an  etiological  factor  in  acute  cerebral 
palsies  has  given  rise  to  some  discussion.  Freud  and  Rie  are  of  the  opinion 
that  convulsions  denote  the  onset  of  the  cerebral  process,  but  that  they  never 
hold  a  causal  relation  to  the  palsy  which  results  from  the  cerebral  lesion.  I 
grant  that  in  a  very  large  number  of  cases  this  statement  of  the  German 
authors  is  tenable,  but  in  many  others  the  palsy  seems  to  be  a  more  or  less 
immediate  result  of  the  convulsive  seizure.  This  view  has  been  urged  by 
Osier  and  myself.  Anyone  who  has  observed  the  marked  disturbances  of 
circulation  at  the  acme  of  convulsions  can  readily  conceive  how  easily  a 
blood-vessel  could  burst  during  this  period  as  a  result  of  excessive  stasis. 
This  does  occur,  moreover,  not  only  in  children,  but  even  in  adults.  I  have 
the  brain  of  a  girl  aged  seventeen,  who  had  had  chronic  epilepsy  for  years,  but 
whose  general  health  was  not  affected  by  the  convulsive  seizures;  after  the 


438 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


last  attack  which  she  had  she  became  somnolent,  comatose,  and  died  after 
three  days.  Her  brain  was  covered  by  a  large  subpial  extravasation,  which 
almost  completely  covered  the  entire  left  hemisphere  and  part  of  the  right. 
If  such  an  occurrence  can  take  place  in  the  brain  of  a  girl  of  seventeen 
there  is  no  sufficient  reason  why  similar  accidents  of  lesser  extent  should 
not  happen  frequently  in  the  case  of  children.  Ashby,  not  long  ago  de- 
scribed the  brain  of  a  child,  twelve  years  of  age,  in  which  a  number  of  old 
cysts  were  found  which  were  probably  the  result  of  hemorrhages  occurring 
during  convulsions.  There  is  clinical  evi- 
dence also  which  cannot  be  neglected 
which  tends  to  show  that  the  convulsions 
constitute  a  decided  danger  to  the  child, 
and  mark  a  turning-point  in  its  entire 
career.  I  have  still  under  my  observation 
a  child,  now  eighteen  years,  which  I 
knew  from  the  time  of  its  birth,  and  which 
was  entirely  normal  until  the  age  of  fifteen 
months.  It  had  begun  to  stand,  to  walk 
a  little,  and  had  acquired  some  speech. 
It  was  in  every  way  a  healthy  and  men- 
tally vigorous  child.  At  that  age  it  was 
stricken  down  with  chicken-pox,  and  had 
a  single  convulsive  seizure,  with  a  marked 
rise  of  temperature  at  the  onset  of  this 
acute  infectious  trouble.  From  that  time 
on  the  child's  mental  condition  changed  ; 
it  lost  its  speech,  and  to  this  day,  although 
it  has  grown  physically,  its  mental  condi- 
tion is  that  of  complete  idiocy.  It  would 
be  a  very  remarkable  instance  indeed  if 
an  acute  cerebral  process  had  come  on 
at  exactly  the  same  time  as  the  chicken- 
pox. 

The  Form  of  Palsy. — Hemiplegia, 
diplegia,  and  paraplegia  are  the  usual 
forms  of  cerebral  paralysis  in  children. 
Monoplegia,  which  we  might  expect  on 
theoretical  grounds,  is,  as  a  matter  of  fact, 
extremely  rare.    (Figs.  105-108.) 

The  leg  evidently  recovers  very  much  more  quickly  than  the  arm,  as  in 
adult  hemiplegia,  and  for  this  reason  it  is  a  very  great  rarity  to  find  a  mono- 
plegia of  the  leg  with  few  symptoms  in  the  arm  of  the  same  side.  Under 
the  heading  diplegia  we  may  classify  all  those  cases  in  which  both  halves  of 
the  body  have  been  involved,  and  it  is  better  to  attach  the  greatest  impor- 
tance to  this  bilateral  character  of  the  palsy,  even  though  the  palsy  be  incom- 
plete, for  the  bilateral  affection  points  to  a  double  brain  lesion,  and  that  is 
the  salient  point  in  every  such  patient.     In  diplegia  the  legs  may  often  be 


Fig.  107.  —  Congenital  Diplegia  — 
"  Frog  Girl."  Double  talipes 
equino  varus  ;  athetosis  of  left 
hand ;  right  arm  also  weak ;  in- 
telligence good. 


INFANTILE   CEREBRAL   PALSIES.  439 

much  more  affected  than  the  arms,  and  some  authors  might  be  inclined  to 
classify  such  palsies  under  the  category  of  paraplegia.  In  a  number  of  in- 
stances there  has  been  a  very  complete  spastic  paraplegia  associated  with 
athetoid  or  choreic  disturbances  of  motion  in  the  upper  extremities.  This 
proves  that  the  upper  extremities  were  at  one  time  involved,  and  for  this  rea- 
son I  prefer  to  denote  such  conditions  as  an  incomplete  diplegia,  or  diplegia 
with  partial  recovery.  Diplegias  or  paraplegias,  in  short  double  cerebral 
palsies,  are,  in  the  large  majority  of  instances,  due  to  a  prenatal  lesion,  or  to 
traumatism  during  labor,  but  I  have  been  able  to  satisfy  myself  over  and 
over  again  that  both  diplegias  and  paraplegias  do  occur  in  acute  cerebral 
cases,  and  for  this  reason,  in  addition  to  others,  I  must  protest  against  Strum- 
pell's  views  of  making  infantile  hemiplegia  synonymous  with  the  acute  cere- 
bral palsy  of  children.* 

The  involvement  of  the  face  is  a  matter  of  some  interest.  In  children  that 
are  observed  long  after  the  onset  of  the  disease  the  face  appears  to  be  entirely 
normal,  but  in  fully  twenty  per  cent,  of  the  acute  forms  which  I  have  had  an 
opportunity  of  examining,  facial  palsy  was  present  in  the  earlier  stages  of  the 
disease.  I  have  never  observed  a  double  facial  palsy  in  cases  of  diplegia. 
The  reason  of  this  is  not  far  to  seek,  for  the  peculiar  position  of  the  facial 
centre  is  such  that  freshly  exuded  blood  would  not  be  apt  to  adhere  to  this 
part  of  the  brain.  Since  the  publication  of  my  own  articles  on  this  subject, 
and  those  of  Freud  and  Rie,  Koenig  has  called  attention  to  the  occurrence 
of  mimetic  facial  palsies  in  children.  The  cases  which  he  cites  are  very  con- 
vincing, but  this  special  form  of  facial  palsy  is  surely  very  rare,  or  else  it 
would  scarcely  have  escaped  the  observation  of  so  many  authors. 

Aphasia  is  at  times  associated  with  acute  cerebral  palsy.  It  is  invariably 
motor,  not  sensory  in  character.  Of  course  aphasia  will  be  developed  only  in 
those  children  who  have  acquired  articulate  speech  before  the  onset  of  the 
cerebral  palsy.  This  excludes  from  the  list  all  diplegias  and  paraplegias 
which  have  come  on  before  or  during  birth.  There  may  be  defective  de- 
velopment of  speech,  but  no  aphasia  in  any  true  sense  of  the  word.  I  have 
the  records  of  at  least  seventeen  children  with  hemiplegia  and  undoubted 
aphasia.  Of  these  seventeen,  ten  occurred  with  right  hemiplegia,  and  seven 
with  left  hemiplegia.  Eight  of  these  seventeen  were  observed  by  me  in  pri- 
vate practice,  and  of  these  eight,  five  have  been  distinctly  aphasic,  and  three 
of  the  five  were  cases  of  left  hemiplegia.  It  is  of  interest  to  note  the  relatively 
large  proportion  of  aphasia  in  children  with  left  hemiplegia.  As  we  grow 
older  the  left  hemisphere  evidently  obtains  the  upper  hand,  and  after  the 
earlier  years  of  life  we  become,  for  all  practical  purposes,  more  and  more 
left-brained.  Bernhardt  has  come  to  the  same  conclusion  that  aphasia  in 
children  accompanies  right  as  well  as  left  hemiplegia.  Aphonia  has  been 
recorded  by  some. 

Inasmuch  as  the  cerebral  palsies  are  due  to  lesions  which  involve  other 

parts  of  the  brain  as  well  as  the  motor  areas,  it  is  not  unreasonable  to  look 

for  additional  symptoms  in  these  cases.     Thus  Freud  was  the  first  to  call 

attention  to  the  fact  that  hemianopsia  was  at  times  associated  with  the  brain 

*  The  experience  of  more  recent  years  has  fully  corroborated  these  opinions. 


440 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


palsies  of  children.  Oppenheim,  Peritz,  and  some  French  observers  have 
noted  the  occurrence  of  peculiar  reflex  actions — "  Feed  reflex  "  ;  also  an  in- 
creased "  motor  acoustic  reaction."  The  latter  is  identical  with  the  "  hypera- 
cusis "  to  which  I  directed  attention  many  years  ago.  (See  Amaurotic 
Family  Idiocy.) 

The  rigidities  and  contractures  deserve  especial  no- 
tice. Some  degree  of  con- 
tracture is  present  in  fully 
seventy-five  per  cent,  of  the 
diplegias  and  paraplegias. 
The  contractures  occur 
early  after  the  onset  of  the 
pals)7,  and  evidently  are  de- 
veloped much  more  easily 
than  in  the  cerebral  palsies 
of  the  adult.  It  is  interest- 
ing in  this  connection  to  re- 
fer to  the  case  of  Cotard,  in 
which  the  palsy  was  due  to 
an  intra-uterine  lesion,  and 
the  child  was  born  with  con- 
tractured  extremities.  The 
contractures  may  vary  great- 
ly in  degree  and  in  the  num- 
ber of  joints  affected.  The 
flexors  and  pronators  of  the 
arm,  the  flexors  of  the  legs 
and  of  the  feet,  are  most  fre- 
quently affected.  In  the  cases 
of  diplegia  and  paraplegia 
there  is,  in  addition,  a  con- 
tracture of  the  adductors  of 
the  thighs,  which  is  respon- 
sible for  the  peculiar  cross- 
legged  position  of  the  legs 
and  for  the  cross-legged  progression  if  the  child  is  at  all  able 
to  walk.  All  these  contractures  give  the  child  a  characteris- 
tic position  and  gait  by  which  we  can  recognize  the  trouble 
at  first  sight.  Pes  equinus  or  pes  equino  varus  is  the  most 
common  deformity  of  the  foot.     In  a  few  cases  an  equino 


Fig.  108.  —  Case  of  Spastic  Diplegia, 
Attempt  to  walk  ;  cross-legged  pro- 
gression ;  rigidity  and  paralysis  of  legs 
and  of  right  upper  extremity  ;  left  up- 
per extremity  weak. 


INFANTILE    CEREBRAL   PALSIES.  44 1 

valgus  is  present.  If  the  upper  extremity  is  contractured 
the  arm  is,  as  a  rule,  in  close  juxtaposition  to  the  trunk, 
flexed  at  the  elbow,  and  the  hand  is  generally  in  a  position 
of  extreme  flexion,  the  fingers  often  being  firmly  pressed 
into  the  palm  of  the  hand.     (See  Fig.  106.) 

The  gait  varies  much  and  is  dependent  both  upon  the  paralysis  and  the 
degree  of  contracture.  Many  cases  of  hemiplegia  in  children  have  exactly 
the  same  walk  as  the  adult  hemiplegic  patients  have,  but  the  peculiar  cross- 
legged  progression  in  cases  of  diplegia  and  paraplegia  is  characteristic  of 
these  infantile  palsies,  and  has  no  counterpart  in  the  cerebral  palsies  of  the 
adult.  In  one  case  the  contractures  of  the  hip  and  knees  were  so  extreme 
that  the  girl,  who  was  otherwise  well  developed  and  bright,  could  walk  only 
by  skipping  in  the  manner  of  a  frog.  She  was  baptized  in  my  clinic  as  the 
"Frog  girl."     (Fig.  107.) 

The  reflexes  are  almost  invariably  exaggerated,  as  can  be  expected  from 
the  fact  that  the  lesion  is  in  the  first  division  of  the  motor  tract.  In  a  few 
instances  only  have  I  found  the  knee-jerk  either  normal  or  diminished. 
Babinski's  reflex  can  be  elicited  in  most  cases,  and  Oppenheim's  tibial  reflex 
in  some.  (Dorsal  flexion  of  foot  and  toes  following  upon  stroking  of  inner 
side  of  leg.)  In  one  child  seen  at  my  clinic  the  reflexes  were  increased  in 
the  upper  extremities,  and  decreased  in  the  lower,  although  the  case  was  one 
of  right  hemiplegia ;  on  closer  examination  we  discovered  that  two  years  after 
the  onset  of  the  cerebral  palsy  the  child  suffered  an  attack  of  polio-myelitis 
involving  the  right  leg.  This  is,  by  the  way,  the  only  patient  I  have  seen 
with  a  spinal  lesion  complicating  cerebral  disease.  In  some  cases  the  reflexes 
appear  to  be  absent,  but  this  is  generally  due  to  a  very  marked  contracture 
of  the  opposing  muscles,  which  do  not  permit  the  excursion  of  the  leg.  Un- 
der such  circumstances,  however,  even  though  the  knee-jerk  be  wanting,  the 
anterior  thigh  muscle  can  be  seen  to  contract  upon  the  tapping  of  the  tendon. 
The  ankle  clonus  and  the  triceps  reflex  are  often  inhibited  for  the  reasons  just 
stated.  In  other  individuals,  the  reflexes  are  usually  so  much  exaggerated 
that  a  single  tap  of  the  tendon  is  sufficient  to  produce  clonic  contractions  of 
the  muscle. 

The  post-paralytic  disturbances  of  motion  constitute  a  very 
characteristic  feature  of  cerebral  palsies  in  children,  and 
they  follow  with  much  greater  regularity  upon  the  infantile 
cerebral  lesions  than  they  do  upon  the  brain  lesions  of  the 
adult.  That  they  are  by  no  means  uncommon  in  the  brain 
lesions  of  the  adult,  I  had  occasion  to  observe  several  times, 
notably  in  an  old  woman,  with  a  lesion  in  the  crus,  whose 
paralyzed  arm  performed  the  most  violent  ataxic  move- 
ments. In  children  these  post-paralytic  disturbances  of 
motion  occur  in  fully  one-third  of  all  the  cases. 


442  THE  NERVOUS  DISEASES   OF  CHILDREN. 

From  a  collection  of  statistics  based  upon  one  hundred  and  fifty-six  cases 
of  hemiplegia,  and  thirty-nine  cases  of  diplegia,  it  was  evident  that  athetoid, 
choreiform,  and  associated  movements,  are  the  most  frequent  disturbances 
to  be  noted.  The  choreiform  and  athetoid  can  generally  be  differentiated 
from  one  another,  but  in  some  cases  there  may  be  a  combination  of  both. 
The  athetoid  movements  are  of  especial  interest,  inasmuch  as  they  are  ob- 
served almost  invariably  in  connection  with  the  cerebral  palsies,  and  it  is 
questionable  whether  athetosis  ever  occurs  as  an  independent  disease,  as  was 
claimed  by  Hammond,  who,  however,  deserves  all  the  credit  for  the  first  vivid 
description  of  these  peculiar  movements. 

The  associated  movements  are  often  developed  to  a  startling  degree,  the 
paralyzed  hand  imitating  all  the  movements  of  the  normal  hand.  Thus  a 
patient  who  is  asked  to  button  or  unbutton  his  clothes,  will  imitate  all  the 
movements  of  the  sound  hand,  by  the  paralyzed  hand,  although  the  latter 
may  be  held  in  mid-air. 

The  choreiform  movements  occurring  after  cerebral  pal- 
sies deserve  some  special  notice,  for  such  conditions  are  at 
times  mistaken  for  cases  of  ordinary  chorea.  I  have  been 
consulted  in  not  a  few  instances  for  what  was  supposed  to  be 
incurable  St.  Vitus's  dance,  which  on  examination  has  proved 
to  be  a  form  of  infantile  cerebral  palsy,  in  which  the  palsy 
had  largely  disappeared,  but  the  choreiform  movements 
were  left  as  evidences  of  the  former  more  serious  dis- 
turbance. In  such  children  the  presence  of  paralysis  or  of 
contractures,  however  slight,  and  the  exaggeration  of  the 
reflexes  in  the  choreic  extremity,  will  be  sufficient  to  indi- 
cate the  nature  of  the  choreic  movements. 

Atrophy  of  the  muscles  plays  an  entirely  negative  part  in  the  vast  majority 
of  infantile  spastic  palsies.  Quincke,  Borgherini,  Darkschewitsch,  Eisen- 
lohr,  and  others,  have  proved  beyond  a  doubt  that  a  cerebral  muscular  atro- 
phy does  at  times  occur,  but  it  is  still  questionable  whether  this  is  due  to 
a  lesion  of  the  trophic  centres  in  the  brain,  or  whether  the  spinal  gray  mat- 
ter has  not  in  some  way  been  involved.  I  have  observed  a  considerable  atro- 
phy of  the  muscles  in  a  few  cases  of  diplegia  and  paraplegia.  In  one  patient 
with  paraplegia  the  atrophy  was  so  extreme  that  the  case  would  have  ap- 
peared to  have  been  one  of  spinal  palsy  if  the  presence  of  the  reflexes  and 
the  electrical  reactions,  as  far  as  any  could  be  elicited,  together  with  the  en- 
tire history  of  the  disease,  had  not  pointed  unmistakably  to  a  cerebral  lesion. 
While  a  true  muscular  atrophy  does  not  occur;'  as  a  rule,  the  affected  limb  re- 
mains stunted  in  growth,  and  after  a  few  years  is  considerably  shorter  than 
its  fellow  of  the  opposite  side.  Ibrahim  and  others  have  reported  hyper- 
trophy of  the  paralyzed  extremities,  together  with  athetosis,  but  it  has  not 
been  my  good  fortune  to  see  more  than  one  such  case. 


INFANTILE    CEREBRAL   J'ALSIES. 


443 


Remak  has  reported  a  case  of  luxation  of  the  shoulder-joint  in  an  infantile 
cerebral  hemiplegia,  which  he  attributes  to  unequal  innervation  of  the  mus- 
cles surrounding  the  shoulder-joint. 

A  distinct  asymmetry  of  the  body  is  present  in  many  of  the  cerebral  pal- 
sies, and  to  this  we  may  add  the  asymmetric  development  of  the  skull,  which 
has  been  shown  to  be  present,  by  the  careful  measurements  of  Peterson  and 
Fisher,  in  the  majority  of  the  subjects  of  infantile  spastic  hemiplegia ;  but  the 
skulls  of  such  children  are  often  not  only  asymmetrical,  but  also  deformed  in 
various  ways.  Some  of  them  are  microcephalic,  others  leptocephalic,  doli- 
chocephalic, etc.  Microcephalus  is  frequent  enough  to  justify  me  in  caution- 
ing the  surgeon  who  may  be  ready  to  operate  upon  all  such  skulls  that  a 

small  skull  may  harbor  a  dis- 
eased brain.  Ibrahim  has  writ- 
ten on  the  association  of  micro- 
cephalus with  infantile  spastic 
diplegia,  and  believes  that  this 
association  points  to  the  pre- 
natal origin  of  these  diplegias. 

The  most  serious  of 
all  the  symptoms  asso- 
ciated with  infantile 
cerebral  palsy  is  epi- 
lepsy. A  number  of 
children  have  been 
brought  to  me  with  the 
diagnosis  of  epilepsy, 
pure  and  simple,  in 
which  this  condition 
was  the  outcome  of 
the  same  cerebral  dis- 
turbance which  gave  rise  to  the  palsy.  The  pals}'  may  dis- 
appear, or  may  be  so  slight  as  to  give  little  inconvenience 
to  the  child,  whereas  the  epilepsy  unfortunately  remains. 
This  association  is  so  common  that  I  am  inclined  to  the  view 
that  a  fair  percentage  of  the  cases  of  supposed  genuine 
epilepsies  may  be  attributed  to  cerebral  lesions  which  oc- 
curred in  early  childhood,  and  have  given  rise  to  cerebral 
palsies.  I  am  glad  to  see  that  this  view  has  been  quoted 
approvingly  by  a  number  of  authors,  among  others  by 
Freud,  who  has  unquestionably  given  this  entire  subject 
the  most  careful  study.  A  single  case  will  bring  out  the 
truth  of  this  very  clearly. 


Fig.  109.  — A  Child  with  Congenital  Diplegia  and 
a  Microcephalic  Skull  ;  Strabismus  and  Idiocy. 


444  THE  NERVOUS  DISEASES   OF  CHILDREN. 

Some  years  ago  I  was  asked  to  see  a  girl,  seventeen  years  of  age.  A 
number  of  able  physicians  had  treated  her  for  genuine  epilepsy.  Upon  ex- 
amination of  the  girl  I  was  astonished  to  find  that  the  left  extremities  were 
weaker  than  the  right,  and  that  the  deep  reflexes  of  the  left  upper  and  lower 
extremities  were  considerably  exaggerated.  Upon  questioning  the  mother 
closely  I  discovered  that  four  years  previously  the  girl  had  had  a  slight  apo- 
plectic seizure,  after  which  the  convulsions  first  appeared.  The  palsy  had 
diminished  to  such  an  extent  that  everyone  would  have  considered  the  gait 
of  the  girl  entirely  normal.  Her  mental  condition  was  impaired  by  the 
enormous  doses  of  bromide  which  had  been  administered  in  the  hope  of 
curing  the  epilepsy.  Recognizing  its  organic  origin,  I  decided  to  discon- 
tinue the  bromides,  and,  as  a  result,  the  epilepsy  is  no  worse  and  no  better ; 
but  the  girl's  mind  has  considerably  improved,  and  the  anaemia,  which  was 
aggravated  by  the  administration  of  the  bromides,  has  been  considerably 
diminished. 

The  first  epileptic  attacks  associated  with  a  cerebral  palsy  may  occur  very 
soon  after  the  onset  of  the  disease,  and  may  be  repeated  a  number  of  times 
during  the  first  week  or  two.  After  this  a  lull  sets  in,  but  unfortunately  the 
attacks  often  recur  after  several  months,  sometimes  after  a  year  or  more, 
when  it  is  positive  that  a  chronic  epilepsy  has  been  developed.  According  to 
my  own  statistics,  fully  forty-five  per  cent,  of  all  the  infantile  cerebral  palsies 
develop  epilepsy.  This  occurs,  furthermore,  in  about  fifty  per  cent,  of  the 
cases  of  hemiplegia,  in  about  thirty  per  cent,  of  all  forms  of  diplegia,  and  in 
about  thirty-six  per  cent,  of  patients  with  paraplegia.  I  have  furthermore 
observed  that  the  epilepsy  does  not  necessarily  develop  according  to  the 
severity  of  the  palsy,  for  some  of  the  worst  cases  of  epilepsy  I  have  seen  have 
been  observed  in  cases  in  which  the  palsy  was  extremely  slight.  The  corti- 
cal origin  of  infantile  cerebral  palsies  is  sufficient  reason  for  the  more  fre- 
quent development  of  epilepsy  after  infantile  than  after  adult  cerebral  palsies  ; 
and  if  the  character  of  the  palsy  is  such  that  the  lesion  in  the  child  can  be 
proved  to  be  capsular  rather  than  cortical,  the  prognosis  as  regards  epilepsy 
may  be  considered  favorable.  Jacksonian  epilepsy  is  present  in  some  of  the 
palsies,  and,  if  present,  can  easily  be  explained  on  physiological  grounds,  but, 
as  a  matter  of  fact,  it  is  not  observed  as  commonly  as  one  would  suppose, 
for  the  simple  reason  that  the  majority  of  patients  have  been  examined  after 
several  years  have  elapsed,  and  the  unilateral  order  of  convulsions  has  been 
superseded  by  a  general  convulsion.  Koenig  has  given  close  study  to  this 
part  of  the  subject. 

Idiocy  is  developed  very  often  with  epilepsy  and  with 
some  forms  of  cerebral  palsy.  Every  possible  variation  of 
mental  deficiency  may  be  present,  from  slight  mental  en- 
feeblement  to  a  complete  idiocy.  The  severer  forms  are 
oftener  present  in  the  cases  of  diplegia  and  paraplegia  than 
in  hemiplegia,  and  naturally  enough,  for  in  the  former  there 
is  a  double  lesion  or  disease  of  both  halves  of  the   brain 


INFANTILE    CEREBRAL   PALSIES.  445 

which  will  disturb  the  mental  development  far  more  than  a 
unilateral  lesion  would. 

I  have  found  idiocy  present  in  thirty-five  per  cent,  of  all  diplegias,  and  in 
sixty  per  cent,  of  all  paraplegias,  while  it  occurred  in  but  thirteen  per  cent, 
of  the  subjects  of  hemiplegia.  It  was  this  very  high  percentage  of  idiocy  in 
cases  of  diplegia  and  paraplegia  that  forced  the  conclusion  upon  me,  some 
years  ago,  that  these  congenital  paraplegias  must  be  of  cerebral  rather  than  of 
spinal  origin.  There  is  no  telling  in  advance  whether,  in  a  given  case,  idiocy 
will  develop  or  not,  for,  unfortunately,  relatively  slight  paralysis  is  sometimes 
complicated  by  severe  idiocy.  Marie  claims  that  there  is  no  disturbance  of 
intelligence  in  cases  of  double  athetosis.  I  must  insist  that  there  are  excep- 
tions even  to  this  rule.  The  frontal  lobes  are  supposed  to  be  silent  parts  of 
the  brain,  but  the  defect  in  intelligence  tells  a  sadder  tale  of  its  important 
functions  than  the  palsy  does  of  the  functions  of  the  motor  centres. 

Various  attempts  have  been  made  to  classify  the  cere- 
bral palsies  of  children,  and  to  separate  them  into  distinct 
clinical  groups.  The  classification  according  to  the  period 
of  development,  which  we  have  adopted,  is,  on  the  whole, 
the  most  practical  one.  The  palsies  due  to  intra-uterine 
disease  or  to  arrest  of  development,  and  those  due  to 
traumatism  during  labor,  give  a  distinct  history  of  early 
onset  of  all  the  symptoms.  In  these  children  bilateral  pal- 
sies (diplegia  and  paraplegia)  are  more  common  than  in  the 
acute  cases,  and  there  is  often  a  very  decided  defect  in 
mental  development.  The  paralysis  is  not  necessarily  com- 
plete ;  at  times  it  is  a  simple  paresis,  at  other  times  rigidi- 
ties and  contractures  are  more  prominent  than  the  palsies 
are.  English  and  American  authors  have  not  drawn  any 
sharp  clinical  lines,  as  all  these  forms,  although  they  may 
vary  a  little,  seem  to  merge  into  one  another,  and  are  evi- 
dently due  to  similar  morbid  processes.  Little  distinguished 
between  rigidities  and  palsies,  and  several  German  authors 
have  adhered  to  this  distinction ;  but  one  of  the  ablest  of 
them,  Freud,*  has  practically  adopted  the  American  point 
of  view.  Rigidity,  contracture,  choreic  or  athetoid  move- 
ments, paresis,  paralysis  (unilateral  or  bilateral),  constitute 
a  graduated  series  of  disturbances  due  to  cerebral  lesions 
in  children.     Each  one,  or  a  combination  of  all  of  them,  may 

*  This  author  subdivides  the  diplegias  into  four  groups  :  1.  Universal  spastic  rigidity; 
2.  paraplegic  rigidity  ;  3.  bilateral  hemiplegia  ;  4.  bilateral  chorea  and  bilateral  athetosis. 


446  THE  NERVOUS  DISEASES   OF  CHILDREN. 

be  present  in  a  given  case,  and  may  vary  according  to  the 
site  and  intensity  of  the  lesion.  The  best  proof  that  all  these 
disturbances  of  motility  are  closely  related  is,  that  in  certain 
cases  of  diplegia  there  is  paralysis  in  the  lower  extremities 
and  athetosis  in  one  or  both  upper  extremities.  Double 
athetosis  is  evidently  a  condition  closely  analogous  to  the 
double  hemiplegia,  with  less  paralysis  than  in  other  cases. 
Freud  showed  that  there  was  a  form  of  acute  cerebral  palsy 
with  little  palsy  but  distinct  chorea ;  he  described  this  con- 
dition as  one  of  choreiform  paresis,  and  recognized  an  early 
and  a  late  form.  Unilateral  and  bilateral  chorea  and  athe- 
tosis may  be  developed  in  the  place  of  a  palsy,  or  the  palsy 
may  come  first  and  the  chorea  and  athetosis  may  represent 
post  hemiplegic  disturbances  of  motion. 

The  diplegias  and  paraplegias  are  generally  due  to  ar- 
rest of  development  or  to  abnormal  birth  conditions.  (Some 
distinctly  hereditary  and  family  affections  allied  to  these 
forms  will  be  discussed  at  the  close  of  this  chapter.)  Charcot, 
Marie,  Dejerine,  van  Gehuchten,  and  Spiller  insist  that  con- 
genital spastic  paraplegia  may  be  a  spinal  affection  and  due 
to  defective  development  of  the  pyramidal  tract.  For  a  time 
Ross  adhered  to  a  similar  view  ;  but  the  defect  surely  in- 
volves the  brain  in  a  large  majority  of  instances,  or  else  we 
could  not  account  for  the  high  percentage  of  idiocy  among 
children  with  congenital  paraplegia.  It  is  in  this  group  of 
diseases  that  thoroughly  satisfactory  post-mortem  examina- 
tions are  needed.  One  case  of  Foerster  and  one  of  my  own 
have  been  relied  upon.  Recently  Dejerine  has  made  an 
interesting  contribution  to  this  subject. 

Morbid  Anatomy. — The  study  of  the  morbid  lesions 
underlying  these  cerebral  palsies  in  children  will  be  facili- 
tated very  much  by  considering  separately  the  three  chief 
groups  of  cases.  A  reference  to  the  following  table  will 
show  that  in  the  prenatal  cases  large  cerebral  defects  are 
often  found.  Porencephaly  is  present  in  its  widest  sense. 
Half  or  even  more  of  an  entire  hemisphere,  or  of  both 
hemispheres,  may  be  wanting,  or  a  considerable  portion  of 
one  hemisphere  may  be  poorly  developed  ;  in  such  cases,  too, 
as  a  rule,  the  palsy  which  exists  is  but  one  of  a  long  series  of 
symptoms,  among  which  idiocy  is  by  far  the  most  promi- 


INFANTILE    CEREBRAL   PALSIES. 


447 


nent.  If  it  were  not  for  the  fact  that  porencephalic  defects 
occur  more  frequently  in  the  motor  areas  of  the  brain  than 
in  any  other  part,  we  would  scarcely  be  entitled  to  associate 
porencephaly  more  especially  with  these  cerebral  palsies. 

Classification  of  Infantile  Cerebral  Palsies. 


Groups. 


I.  Paralyses  of  intra-uterine 
onset 


II.  Birth  Palsies 


Morbid  Lesion. 


Large  cerebral  defects.     (Porencephaly). 
Defective  development  of  pyramidal  tracts  (p.  446). 
Agenesis  corticalis.     (Highest  nerve  elements  in- 
volved.) 


Meningeal  hemorrhage,  rarely  intra-cerebral 
hemorrhage.  Later  conditions :  Meningoen- 
cephalitis chronica,  sclerosis,  and  cysts  ;  partial 
atrophies. 


HI.  Acute  (acquired)  Palsies.. 


Hemorrhage  (meningeal,  and  rarely  intra-cere- 
bral) ;  thrombosis  (from  syphilitic  endarteritis 
and  in  marantic  conditions  ;  embolism.  Later 
conditions :  Atrophy,  cysts,  and  sclerosis  (dif- 
fuse and  lobar). 

Meningitis  chronica. 

Hydrocephalus  (seldom  the  sole  cause). 

Primary  encephalitis;  polio  -  encephalitis  acuta 
(Strumpell). 


In  addition  to  these  large  defects  in  cerebral  develop- 
ment, other  changes  occur  in  the  brain  which  are  not  quite 
as  conspicuous,  but  are  fully  as  effective  in  the  way  of  pro- 
ducing serious  symptoms  and  serious  conditions  during  life.* 
This  condition  has  been  properly  designated  "  agenesis  cor- 
ticalis." It  implies  a  defective  development  of  the  cellular 
elements  of  the  cortical,  and  particularly  of  the  pyramidal, 
cells,  and  is  not  restricted  to  any  one  part  of  the  cortex,  but 
involves  all  parts  of  the  hemispheres  about  equally.  This 
condition  is  present  in  central  amaurotic  idiocy,  associated 
with  similar  changes  in  the  gray  matter. 

The  morbid  lesions  in  birth  palsies  have  been  satisfacto- 
rily cleared  up  within  the  past  few  years.  We  owe  much  . 
of  this  knowledge  to  the  successful  demonstration  by  Dr. 
Sarah.  McNutt  of  a  case  in  which  there  was  wide-spread  men- 
ingeal hemorrhage  in  consequence  of  traumatism  during 
labor.  (Fig.  no.)  Dr.  McNutt  furnished  positive  proof 
of  a  condition  which  Little  suspected  years  ago,  in  attribut- 


448 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


ing  these  palsies  to  the  difficulties  during  birth.  There 
can  be  no  doubt  but  that  in  a  vast  majority  of  cerebral  birth 
palsies  meningeal  hemorrhage,  more  or  less  diffuse  over 
one  or  both  hemispheres,  is  the  direct  cause  of  the  disease. 

The  failure  to  recognize  this  simple  condition  has  been  due  to  the  fact 
that  the  cases  examined  for  the  purpose  of  determining  the  morbid  states 
underlying  these  palsies  have  lived  for  a  number  of  months,  or  even  a  year, 
and  at  the  end  of  that  time  the  character  of  the  initial  lesion  cannot  be 
recognized.     A  very  instructive  case  of  this  description  is  the  one  referred  to 


Fig.  iio. — Meningeal  Hemorrhage  at  Birth.     Death  on  the  twenty-second  day. 

(McNutt.) 

in  a  previous  chapter  (page  ).  The  child  was  born  asphyxiated  after 
forty-eight  hours  continuous  labor  pains,  and  an  absolutely  dry  labor  at  that. 
From  the  very  first  day  of  its  life  until  the  age  of  six  months,  when  I  first 
saw  the  child,  it  had  innumerable  epileptic  spells.  The  child  died  at  the  age 
of  one  year.  The  autopsy  and  a  careful  microscopic  examination  revealed  a 
chronic  meningo-encephalitis.  This  condition  was  in  all  probability  due  to  a 
meningeal  hemorrhage,  though  the  traces  of  the  hemorrhage  had  so  thor- 
oughly disappeared  that  it  was  difficult  to  prove  this  with  absolute  certainty. 
In  some  instances  an  original  meningeal  hemorrhage,  particularly  if  it  be 
more  or  less  circumscribed,  may  lead  to  the  formation  of  a  limited  atrophy, 
in  which  case  a  condition  resembling  porencephalus  may  be  the  result.  A 
few  years  ago  Kundrat  described  a  condition  of  minute  hemorrhages  from  the 
meningeal  veins,  which  he  considered  to  be  caused  by  a  compression  of  the 
longitudinal  sinus,  in  consequence  of  the  displacement  of  the  parietal  bones. 


INFANTILE   CEREBRAL   PALSIES.  449 

Such  hemorrhages  Kundrat  thought  were  found  rather  frequently  in  the  brains 
of  healthy  children.  It  is  doubtful  whether  we  can  connect  this  form  of 
venous  hemorrhage  with  the  subject  which  we  are  now  considering. 

The  amount  of  damage  done  during  labor  can  be  gauged 
to  a  certain  extent  by  the  symptoms  during  the  first  few 
days  of  life.  If  there  is  wide-spread  meningeal  hemorrhage 
convulsions  set  in  at  a  very  early  period  ;  the  child  is  apt  to 
be  paralyzed  at  once,  and  is  also  in  danger  of  lapsing  into 
coma.  Fortunately  a  number  of  the  severer  cases  die 
promptly  within  a  few  days,  but  others  again  survive,  and 
not  a  few  of  these  become  hopelessly  crippled  for  life.  Ac- 
cording to  the  amount  of  injury  done,  and  according  to  the 
special  parts  of  the  brain  covered  by  the  hemorrhage,  the 
form  of  the  palsy,  the  degree  of  mental  enfeeblement,  and 
the  severity  of  the  epilepsy  will  vary  not  a  little. 

The  morbid  anatomy  of  the  acute  cerebral  palsies  has 
given  rise  to  the  largest  amount  of  discussion.  Hemor- 
rhage, embolism,  and  thrombosis,  the  conditions  which  give 
rise,  in  the  vast  majority  of  cases,  to  the  apoplexy  of  the 
adult,  are  important  factors  also  in  the  causation  of  the  acute 
cerebral  palsies  of  children.  This  conclusion  was  forced 
upon  us  and  others  by  a  careful  review  of  the  autopsies 
made  by  different  authors. 

Peterson  and  myself  analyzed  the  records  of  seventy- 
eight  autopsies  in  infantile  hemiplegia,  as  follows  : 

Lesions.  Number  of  Cases. 

Atrophy,  sclerosis,  and  cysts  (terminal  conditions) 40 

Porencephalus 2 

Hemorrhage 23 

Embolism 7 

Thrombosis 5 

Tubercle 1 

Total 78 

In  35  cases  of  hemiplegia  hemorrhage  occurred  in  23, 
embolism  in  7,  and  thrombosis  in  5  cases.  Professor  Osier, 
who  examined  90  brains,  found  a  vascular  lesion  in  16  only, 
and  among  these  16,  7  were  due  to  hemorrhage,  and  9 
to  embolism  ;  but  we  must  not  forget  that  a  large  number 
of  the  cystic  conditions  may  be  due  to  hemorrhage  or  em- 


450  THE   NERVOUS  DISEASES    OF   CHILDREN. 

bolism,  and  that  many  a  case  which  is  quoted  as  typical 
atrophy  or  porencephalus  may  also  belong  to  the  same 
category.  There  has  been  some  little  difference  of  opinion 
among  recent  writers  on  this  subject  with  regard  to  the 
relative  frequency  of  these  various  conditions.  Some  are  in 
favor  of  the  greater  frequency  of  embolism  ;  others,  like  my- 
self, believe  that  hemorrhages  are  most  commonly  the  cause 
of  these  conditions ;  while  according  to  Gowers  thrombo- 
sis of  the  superficial  veins  is  a  very  noteworthy  initial  lesion. 
The  probability  is  that  all  these  accidents  occur,  and  that 


Fig.  ill. — Cyst  formed  by  Softening  of  Brain  Substance,  secondary  to  Obstruction  of 
the  Middle  Cerebral  Artery  beyond  the  First  Branch  (to  Inferior  Frontal  Convolu- 
tion). The  cyst  wall  has  fallen  in  from  escape  of  its  contents.  Child  nineteen 
months  old.  Death  seven  months  after  onset  of  paralysis.  (After  Ashby  and 
Wright) 

there  is  no  satisfactory  reason  for  claiming  more  for  the 
one  than  for  the  other  finding  ;  but  with  regard  to  the  occur- 
rence of  hemorrhages  it  is  fair  to  inquire  what  morbid  con- 
dition of  the  blood-vessel  leads  to  the  frequent  occurrence 
of  hemorrhage  in  children. 

In  my  endeavors  to  elucidate  this  part  of  the  inquiry  I  have  been  compelled 
to  fall  back  upon  the  fatty  degeneration  of  the  blood-vessels  which  Reck- 
linghausen describes  in  his  masterly  Monograph,  and  which  he  claims  is  a 
not  uncommon  finding  in  the  brains  of  children.  For  the  present  we  can  do 
no  better  than  to  adhere  to  this  view  of  Recklinghausen,  and  to  accept  it  as  a 
partial  explanation,  at  least,  of  the  frequent  occurrence  of  hemorrhage.  Some 
cases  are  of  course  explained  by  the  more  delicate  structure  of  the  blood-ves- 


INFANTILE    CEREBRAL    PALSIES. 


451 


sels  in  children  as  compared  with  the  adult.  But  there  is  still  another  striking 
difference  between  these  infantile  and  adult  apoplexies.  In  the  adult  the 
majority  of  hemorrhages  occur  in  the  vicinity  of  the  internal  capsule.  In  the 
child  they  occur  in  or  near  the  cortex.  Mendel  has  shown  that  the  greatest 
pressure  is  exerted  in  the  branches  of  the  middle  cerebral  artery,  and  that 
any  increase  of  pressure  would  naturally  cause  a  rupture  in  one  of  these. 
We  would  have  to  seek  some  other  explanation  for  the  frequent  occurrence 
of  cortical  and  meningeal  hemorrhages,  and  perhaps  they  will  be  sufficiently 
accounted  for  by  the  more  delicate  structure  of  these  vessels  in  children.  Oc- 
casionally intra-cerebral   hemorrhages,  embolism,  and   thrombosis  occur   in 


■r^-^-V-' 


Fig.  112 — Section  through  Portion  of  Motor  Cortex,  removed  by  Dr.  Gerster  during 
an  Operation  for  Localized  Epilepsy,  associated  with  Old  Left  Hemiplegia  in  a  Boy 
Twelve  Years  of  Age.  Specimen  was  stained  according  to  Van  Gieson's  method. 
The  pia,  P,  which  is  greatly  thickened  and  altered,  dips  down  between  two  folds  of 
the  cortex,  C.  B,  increased  number  of  thickened  small  arteries  ;  just  above,  to  the 
right,  a  large  artery  with  thickened  walls.  H  points  to  a  recent  clot.  The  line,  a  b, 
denotes  the  part  of  cortex  examined  under  higher  power  and  represented  in  Fig.  146. 


children  in  exactly  the  same  manner  in  which  they  occur  in  the  adult,  and 
they  give  rise  to  clinical  symptoms  in  nowise  different  from  those  of  ordinary 
apoplexy.  An  interesting  case  of  this  sort  I  had  occasion  to  observe  in  a  boy, 
two  and  a  half  years  of  age,  who  after  a  simple  attack  of  tonsillitis  developed 
right  hemiplegia  with  motor  aphasia,  without  coma  and  without  convulsions. 
The  aphasia  disappeared  after  a  few  days,  and  the  hemiplegia  also  improved; 


452 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


within  a  period  of  a  few  weeks  the  young  boy  was  entirely  well,  and  scarcely 
retained  a  trace  of  the  apoplectic  condition.  There  was  no  history  of  syph- 
ilis in  the  case,  and  the  entire  development  and  retrogression  of  the  symp- 
toms reminded  one  of  an  adult  apoplexy.  The  possibility  of  thrombosis 
could  not  be  excluded,  but  whether  hemorrhage  or  thrombosis,  there  can  be 
no  doubt  about  this,  that  the  lesion  was  intra-cerebral  and  not  cortical,  as  in 
the  majority  of  cases  in  children.  Since  my  own  case  was  published,  De- 
jerine.  in  1891,  reported  three  cases  in 
wnich  there  were  distinct  hemorrhagic 
lesions  in  the  vicinity  of  the  larger  gan- 
glia. 


Heart  disease,  rheumatism, 
scarlet  fever,  and  pneumonia, 
are  the  conditions  which  pre- 
dispose to  embolism.  Throm- 
bosis may  be  suspected  in  cases 
of  children  dying  of  marasmus, 
but  under  such  conditions  the 
existence  of  a  palsy,  is,  as  a 
rule,  overlooked,  and  of  little 
practical  importance. 


Thrombosis  may  also  be  the  result  of 
arterial  changes  due  to  hereditary  syph- 
ilis, but  I  wish  distinctly  to  impress  upon 
the  reader  that  in  these  cases  of  cerebral 
palsies  syphilis  does  surely  not  play  as 
important  a  role  as  is  imputed  to  it  by  a 
few  authors,  who  have  generalized  from 
the  examination  of  one  or  two  cases.* 
Gowers  has  advanced  important  reasons 
for  the  supposition  that  thrombosis  and 
occlusion  of  the  middle  cerebral  veins  are 
of  frequent  occurrence  in  children,  and 
that  some  of  the  cases  of  infantile  hemi- 
plegia would  be  more  likely  to  be  due 
to  this  condition  than  to  embolism  re- 
sulting from  endocarditis.  He  states, 
furthermore,  that  the  thrombosis  cannot 
be  distinctly  demonstrated  post-mortem, 
because   the   thrombosis   is,  as  a  rule, 

continued  into  the  sinuses,  and  a  sinus  thrombosis  would  be  much  more 

striking,  and  would  be  apt  to  conceal  the  venous  thrombosis  which  led  to  it. 

*  I  have  had  no  reason  to  change  these  views. 


Fig.  113.  —  Variously  Degenerated 
Cells  of  the  Cortex  in  the  vicinity 
of  the  line  a  b  of  the  preceding  fig- 
ure. Near  <7,  small  blood-vessels, 
walls  thickened.  Large  pyramidal 
cells  are  misshapen,  and  exhibit 
granular  disintegration.  Cells  di- 
minished in  number.  (Drawn  from 
specimen.) 


INFANTILE    CEREBRAL    PALSIES. 


453 


Whatever  the  initial  lesion  of  an  acute  cerebral  palsy 
may  be,  if  the  patient  survive  a  number  of  years,  secondary 
changes  may  be  set  up  in  the  brain  which  will  successfully 
conceal  the  initial  lesion.  Cysts,  large  areas  of  softening, 
atrophy,  sclerosis  (diffuse  and  lobar),  are  a  few  of  the 
changes  frequently  observed. 

There  is  no  telling  in  advance  of  the  post-mortem  examination,  with  any 
degree  of  certainty,  which  secondary  lesion  will  be  found  in  the  brain  of  a 


Fig.  114. — An  Old  Hemorrhagic  Cyst.  The  cyst  walls  have  been  cut  to  expose  tu- 
mor underneath  (compare  with  Fig.  150).  Right  hemiplegia  at  age  of  six  and  a  half 
years  ;  death  two  years  later. 

child  that  has  been  afflicted  with  an  acute  cerebral  palsy  for  a  number  of 
years.  Cysts  are  so  frequent  that  I  have  in  a  number  of  instances  been  able 
to  predict  the  presence  of  this  condition,  particularly  in  those  cases  in  which 
there  was  little  idiocy  associated  with  the  palsy,  and  in  which  everything 
seemed  to  point  to  an  initial  lesion  of  considerable  intensity,  but  limited  in 
extent.  And  in  several  other  cases  that  came  under  my  own  notice  for  opera- 
tion the  surgeon  found  a  cyst  in  the  motor  area,  which  I  had  predicted  to 
be  the  probable  condition. 

Various  forms  of  sclerosis  are  among  the  most  frequent 
sequelas  of  the  initial  lesions  of  infantile  cerebral  palsies. 


454  THE  NERVOUS  DISEASES   OF  CHILDREN. 

This  sclerosis  evidently  starts  from  a  focal  region  and  rap- 
idly speads  throughout  the  brain.  If  we  bear  in  mind  that 
the  hemispheres  are  traversed  by  innumerable  fibres  which 
are  intimately  connected  with  one  another,  we  can  under- 
stand why  a  sclerosis  should  follow  upon  a  relatively  small 
focus  of  disease,  just  as  secondary  degeneration  follows 
upon  a  lesion  in  the  motor  area,  and  affects  all  the  fibres 
transmitting  centrifugal  impulses.  This  sclerosis,  which 
develops  after  an  initial  lesion,  is  largely  responsible  for  the 
symptoms  which  are  so  frequently  associated  with  these 
palsies.     I  refer  to  idiocy  and  epilepsy. 

In  the  table  given  at  the  beginning  of  this  discussion  I  have,  for  the  sake 
of  completeness,  inserted  chronic  meningitis  as  an  occasional  cause  of  an  ac- 
quired cerebral  palsy.  It  is  not  entirely  accurate  to  attribute  a  cerebral 
palsy  to  a  chronic  meningitis,  but  it  is  better  to  say  that  both  the  chronic 
meningitis  and  the  cerebral  palsy  are  the  result  of  a  cerebro-spinal  or  of  a 
convexity  meningitis  which  occurred  early  in  life,  and  which  the  child  sur- 
vived. These  cases  are  sometimes  to  be  differentiated  from  others  by  the 
persistent  paralysis  of  various  cranial  nerves  in  addition  to  the  paralysis  of 
the  extremities. 

The  reader  may  be  surprised  to  find  polio-encephalitis  acuta  given  as  the 
very  last  morbid  condition  underlying  acute  cerebral  palsy.  StriimpeH's  idea 
was  a  fascinating  one  indeed,  and  it  is  to  be  regretted  that  later  post-mortem 
findings  did  not  support  his  theory.  The  analogy  which  this  author  drew 
between  the  infantile  spinal  paralysis  and  the  infantile  cerebral  palsies  was 
based  upon  clinical  resemblance,  but  we  well  know  that  symptoms  which  are 
scarcely  to  be  differentiated  from  one  another  may  be  due  to  a  variety  of 
morbid  processes.  Strumpell  maintained  that  there  was  a  close  clinical  and 
pathological  relation  between  polio-myelitis  and  polio-encephalitis  acuta. 
More  recently  he  has  modified  his  views,  and  now  claims  that  acute  encepha- 
litis of  the  gray,  as  well  as  of  the  white  matter,  may  constitute  the  basis  for 
these  acute  cerebral  palsies  in  children.  The  actual  proof  which  Strumpell 
offered  for  his  views  was  based  upon  the  occurrence  of  two  cases  in  one 
family,  reported  by  Moebius,  in  which  two  children  were  affected  at  one  and 
the  same  time ;  the  one  with  a  typical  polio-myelitis,  and  the  other  with  a 
spastic  cerebral  palsy  without  atrophy.  This  may  have  been  a  mere  coinci- 
dence, and  further  evidence  must  be  forthcoming  before  we  can  accept  this 
as  proof  of  the  theory.  Strumpell  himself  refers  to  two  cases  in  the  adult  in 
which  the  diagnosis  of  hemorrhage  was  made  during  life,  and  in  which,  at 
the  post-mortem  examination,  acute  encephalitis  was  found.  From  this  we 
may  infer  the  possibility  of  a  similar  occurrence  in  children,  but  until  such  a 
condition  can  be  satisfactorily  established  there  is  no  good  reason  to  accept 
encephalitis  as  more  than  the  rarest  of  all  causes  that  give  rise  to  infantile 
cerebral  palsies.     A  case  of  (supposed  infectious)  encephalitis  of  the  island  of 


INFANTILE    CEREBRAL   PALSIES.  455 

Reil,  reported  by  Felsh,  is  not  convincing.  Lesions  affecting  the  motor  areas 
give  rise  to  a  palsy;  similar  lesions  may  affect  silent  parts  of  the  brain,  and 
the  result  of  such  lesions  may  be  a  general  cerebral  disturbance,  defective  in- 
telligence, or  chronic  epilepsy.  The  fact  has  been  well  emphasized  by  Freud 
who  speaks  of  cases  of  "  cerebral  palsy  without  palsy." 

Differential  Diagnosis. — Infantile  cerebral  palsies 
are  frequently  confounded  with  the  acute  spinal  affections. 
The  cerebral  cases  are  characterized  by  the  hemiplegic, 
diplegic,  or  paraplegic  form  of  paralysis,  by  spastic  rigid- 
ities and  contractures,  by  increase  of  the  deep  reflexes,  by 
the  entire  absence  of  any  considerable  degree  of  atrophy, 
and  by  entirely  normal  electrical  reactions.  Anyone  who 
is  able  to  recognize  the  difference  between  lesions  in  the 
first  and  second  divisions  of  the  motor  tract  will  be  able 
to  distinguish  between  the  cerebral  and  spinal  forms  of  in- 
fantile palsies.  Difficulties  may,  however,  arise  in  the  cases 
of  mild  types  of  cerebral  palsy  in  which  the  spasticity  and 
contractures  may  be  but  slightly  developed,  and  the  re- 
flexes may  not  be  very  much  increased  ;  but  even  under 
such  circumstances  the  hemiplegic  distribution  of  the  palsy 
and  the  entire  absence  of  all  changes  in  the  electrical  reac- 
tions will  help  to  demonstrate  the  cerebral  character  of  the 
disease.  In  other  patients,  the  presence  of  athetoid  or 
choreic  movements  in  one  half  of  the  body,  with  slight 
exaggeration  of  the  reflexes,  will  indicate  a  former  cere- 
bral palsy,  the  paralysis  having  wellnigh  disappeared.  Spi- 
nal and  cerebral  palsies  may  occur  at  different  periods  of 
a  child's  life,  but  I  have  seen  only  one  instance  of  this 
among  the  large  number  of  cases  that  have  come  under  my 
notice.  The  spastic  paraplegia  due  to  traumatism  during 
labor  is  to  be  differentiated  from  hereditary  spastic  paraly- 
sis (see  Chapter  XXL)  and  from  syphilis  of  the  spinal  cord 
(see  Chapter  XVII). 

Although  the  diagnosis  is  simple  enough,  the  knowl- 
edge of  these  infantile  cerebral  palsies  is  still  so  very  imper- 
fect among  physicians  that  cases  coming  under  this  cat- 
egory are  frequently  overlooked.  I  would  particularly 
urge  the  examination  of  patients  with  peculiar  forms  of 
athetosis  or  chorea  in  order  to  determine  whether  these 
are  not  associated  with  some  traces  of  a  preceding  cerebral 


456  THE  NERVOUS  DISEASES   OF   CHILDREN. 

palsy.  If  such  children  exhibit  hemiparesis  with  slight 
defects  in  mental  development,  or  with  occasional  epileptic 
attacks,  the  true  nature  of  the  disease  can  readily  be  dis- 
cerned. 

PROGNOSIS. — In  the  congenital  forms  of  cerebral  palsy 
the  prospects  as  regards  life  and  the  normal  development 
of  the  child  cannot  be  formulated  until  after  a  period  of 
some  weeks  or  months.  It  is  a  fortunate  circumstance  on 
the  whole  that  children  who  come  into  the  world  with  a 
severe  cerebral  lesion  succumb  to  it  at  a  very  early  period, 
but  of  those  who  survive  a  fair  proportion  develop  idiocy 
as  well  as  epilepsy,  and  are  frequently  permanently  crip- 
pled. If  convulsions  occur  during  the  earlier  weeks  of 
life  the  severity  of  the  lesion  may  be  inferred  from  this 
fact.  If  such  a  child,  moreover,  show  no  mental  awakening, 
the  probability  of  more  or  less  complete  idiocy  is  very 
great  indeed.  If,  after  a  few  weeks  or  months,  the  convul- 
sions are  noticeably  diminished,  if  the  child  shows  any  ten- 
dency to  a  tolerably  normal  use  of  its  legs,  and  if  it  begins 
to  take  notice  of  its  surroundings,  a  more  favorable  prog- 
nosis may  be  given.  As  long  as  contractures  do  not  de- 
velop the  child  may  acquire  a  fair  use  of  its  extremities. 
Diplegia  and  paraplegia  are  more  apt  to  be  associated  with 
cerebral  deficiency  and  epilepsy  than  is  unilateral  palsy. 

In  the  acute  cases  there  is  much  uncertainty  for  some 
time  after  the  onset  of  the  attack,  and  no  definite  statement 
is  warranted  regarding  the  permanency  of  the  paralysis,  or 
the  mental  condition  of  the  child  until  several  weeks  have 
elapsed,  when  it  will  be  seen  whether  there  is  any  tendency 
or  not  to  recovery.  If  a  child  with  an  acute  form  of  infan- 
tile hemiplegia  shows  some  improvement  after  a  few  weeks, 
either  in  the  leg  or  in  the  arm,  and  if  the  speech  that  was 
lost  begins  to  return,  there  is  every  reason  to  be  hopeful 
regarding  the  ultimate  outcome  of  the  disease  ;  but  if  weeks 
and  months  pass  without  any  such  favorable  change  the 
probability  of  permanent  crippling  of  the  mind  and  body  is 
very  great.  It  is,  however,  a  matter  of  common  experience 
-that  the  child  may  do  extremely  well  for  a  number  of 
months,  for  a  year,  or  even  longer,  after  an  attack,  when 
convulsions  may  reappear  and  the  epileptic  habit  may  be- 


INFANT  I  Lh    CEREBRAL  PALSJi  457 

come  established.     I  have  paid  considerable  attention   to 

this  point,  and  find  that  among  the  cases  examined  by  me 
epilepsy  occurred  in  fully  forty-five  per  cent,  of  all  the 
cerebral  palsies,  and  nearly  one-half  of  the  cases  of  cerebral 
hemiplegia  developed  epilepsy  at  a  later  period.  It  would 
seem  from  this  that  the  unilateral  brain  lesion  leads  to  epi- 
lepsy more  frequently  than  the  double  lesion  does,  for  ac- 
cording to  the  same  statistics  epilepsy  is  present  in  about 
twenty-nine  per  cent,  of  the  diplegias,  and  in  about  thirty- 
six  per  cent,  of  the  cases  of  paraplegia.  My  own  results 
regarding  the  development  of  epilepsy  are  corroborated  by 
the  statistics  of  Gaudard,  Osier,  and  Wallenberg.  The 
fact  that  fewer  cases  of  diplegia  and  paraplegia  survive 
may  account  for  this  apparent  difference  in  favor  of  the 
double  lesions.  If  a  child  that  has  passed  through  an 
apoplectic  seizure,  and  has  some  form  of  congenital  paralv- 
sis  is  taken  with  convulsions,  it  is  more  than  probable  that 
such  convulsions  are  the  first  of  a  series  that  may  lead  to 
the  development  of  chronic  epilepsy,  and  from  this  time  on 
the  prognosis  becomes  extremely  grave. 

Treatment. — The  treatment  of  infantile  cerebral  pal- 
sies is  not  altogether  devoid  of  interest.  These  diseases 
call  for  deliberate  non-interference  at  the  start,  and  for 
sober  judgment  in  therapeutic  matters  during  the  later 
stages.  The  treatment  of  the  later  stages  will  be  prac- 
tically the  same  whether  a  case  be  congenital  or  acquired. 
If  a  child  but  a  few  days  old  exhibits  a  tendency  to  drow- 
siness or  to  convulsions  its  brain  has  in  all  probability  sus- 
tained a  serious  injury  during  labor.  It  should  be  kept 
quietly  in  its  crib  or  bed,  and  the  greatest  attention  should 
be  paid  to  its  nutrition.  These  children  are  often  unable 
to  swallow  and  unable  to  suckle  at  the  breast,  and  for  this 
reason  must  be  carefully  fed  with  a  spoon.  Milk,  properly 
prepared,  according  to  the  age  of  the  child,  is  the  very  best 
nourishment.  If  there  is  a  tendency  to  convulsions,  or  if 
marked  convulsive  seizures  occur,  the  child  may  be  given 
very  minute  doses  of  bromides,  or  minimum  doses  of  mor- 
phia or  chloral.  A  drop  of  the  solution  of  Majendie,  or  a 
grain  of  chloral  per  rectum,  will  be  quite  sufficient  in  the 
case  of  a  new-born  babe.     If  convulsions  continue  in   spite 


458  THE  NERVOUS  DISEASES   OE   CHILDREN. 

of  these  measures,  inhalations  of  chloroform  may  be  prac- 
tised, however  young  the  child  may  be,  but  6l  course  with 
the  greatest  possible  care.  All  other  measures,  such  as 
counter-irritation,  mustard-baths,  and  the  like,  are  practi- 
cally useless. 

In  the  initial  stages  of  an  acute  cerebral  palsy  the  same 
measures  should  be  employed  which  the  physician  would 
resort  to  in  the  case  of  an  adult  apoplexy,  allowing  always  for 
the  difference  in  the  ages  of  the  patients.  Absolute  rest  is 
the  first  condition  of  treatment.  An  ice-bag  may  be  ap- 
plied to  the  nape  of  the  neck,  or  to  the  head,  and  if  it  do  no 
good,  it  will  at  least  do  little  harm.  The  use  of  ergot  or  of 
the  nitrite  of  amyl,  as  has  been  advocated  in  adult  apoplexies, 
I  cannot  favor.  The  fewer  drugs  that  are  administered  the 
better  it  will  be,  and  the  physician  will  do  well  to  limit 
himself  entirely  to  the  administration  of  calomel  in  doses  suf- 
ficient to  procure  free  purging  of  the  bowels,  and  small  doses 
of  bromide,  which  may  be  given  in  order  to  secure  rest  for 
the  disturbed  brain.  At  a  later  period  the  bromides  may 
be  combined  to  advantage  with  the  iodides,  but  in  every 
case  in  which  the  digestion  or  the  general  nutrition  suffers 
from  the  administration  of  these  drugs,  their  use  should  be 
prohibited  for  the  time  being  ;  for  the  good  that  they  can 
effect  will  not  counterbalance  the  evils  that  follow  upon  a 
gastritis  and  its  attendant  malnutrition. 

As  soon  as  the  symptoms  of  the  initial  period  have 
passed,  the  physician  is  compelled  to  prescribe  some  form 
of  treatment  for  the  paralysis  and  the  other  symptoms  of 
the  disease.  The  paralysis  is  the  natural  result  of  the  lesion, 
and  cannot  of  course  be  removed  by  any  therapeutic  meas- 
ures. It  is  well  to  explain  this  distinctly  to  the  parents 
and  relatives,  and  to  state  at  once  the  chronic  nature  of 
these  troubles,  and  the  length  of  time  that  will  probably 
elapse  before  any  great  change  can  be  expected  to  take 
place.  As  soon  as  the  active  period  of  the  disease  has 
passed,  massage  and  electricity  should  be  applied  to  the 
paralyzed  parts,  but  let  the  brain  severely  alone.  The 
effect  of  electricity  upon  the  circulation  within  the  skull  is 
altogether  too  uncertain  to  justify  us  in  tampering  with  it  in 
any  way.     Electricity  applied  to  the  extremities  can  do  no 


INFANTILE    CEREBRAL   PALSIES.  459 

harm,  and  serves  an  excellent  purpose  as  a  means  of  exer- 
cising parts  that  cannot  be  moved  by  the  will.  As  these 
parts  respond  to  the  faradic  as  well  as  to  the  galvanic  cur- 
rent, I  am  in  the  habit  of  using  the  former  chiefly,  and  of 
using  a  current  strong  enough  to  produce  mild  contrac- 
tions. Powerful  currents  should  be  avoided.  The  electric- 
ity should  be  administered  in  sittings  from  ten  to  fifteen 
minutes  every  day,  or  every  other  day,  and  should  be  given 
by  the  physician,  or  by  an  extremely  competent  nurse 
under  the  guidance  of  the  physician.  If  passive  movements 
are  combined  with  ordinary  friction  the  paralyzed  parts  will 
be  kept  in  a  good  state  of  nutrition,  and  the  tendency  to 
contracture  may  be  overcome. 

As  in  the  cases  of  spinal  paralysis,  so  in  these  cerebral  palsies,  orthopedic 
measures  should  be  resorted  to  as  soon  as  contractures  have  become  perma- 
nently established.  I  have  seen  many  a  child  walk  about  fairly  well  a  few 
weeks  after  the  orthopedic  surgeon  has  been  allowed  to  cut  the  tendons,  and 
to  provide  the  proper  orthopedic  appliances.  Contractures,  if  once  formed, 
are  rarely,  if  ever,  recovered  from  in  spontaneous  fashion,  and  if  it  can  be 
proved  that  the  contractures  are  the  chief  hindrance  to  the  child's  progress  in 
walking,  full  liberty  should  be  given  to  the  orthopedic  surgeon ;  Eulenburg, 
Hoffa,  and  others  have  obtained  good  results  by  tendon-transplantation.  I 
have  been  especially  pleased  with  what  Dr.  Gibney  has  been  able  to  do  in 
several  cases  of  marked  disturbances  of  the  upper  extremities  that  have  oc- 
curred in  cases  of  infantile  cerebral  palsy.  I  refer  in  particular  to  the  persist- 
ent athetoid-choreic  movements  of  the  upper  extremities  so  common  in  these 
cases.  By  the  application  of  some  simple  form  of  restraint  such  post-para- 
lytic movements  may  be  successfully  inhibited.  In  the  case  of  a  young  man, 
seventeen  years  of  age,  who  had  acquired  a  cerebral  palsy  when  he  was  ten 
months  old,  and  who  had  exhibited  very  annoying  athetoid  movements  of 
the  upper  extremities  from  that  time  on,  the  simple  splint  arrangement  which 
prevented  the  possibility  of  these  movements,  and  which  was  worn  for  a 
number  of  months,  was  sufficient  to  inhibit  the  athetoid  movements  altogether ; 
and  in  still  another  case  a  marked  post-hemiplegic  chorea  was  much  im- 
proved, if  not  altogether  inhibited,  by  placing  the  arm  in  a  plaster-of-Paris 
bandage  for  a  period  of  two  months. 

The  treatment  of  the  idiocy  must  be  conducted  on  the  same  lines  as  in 
other  forms  of  idiocy. 

By  far  the  most  important  task  is  the  treatment  of  the 
epilepsy  associated  with  the  palsy.  We  have  stated  else- 
where that  this  epilepsy  is  due  to  the  secondary  changes  in 
the  brain,  which  have  developed  as  a  natural  result  of  the 


460  THE  NERVOUS  DISEASES   OF  CHILDREN. 

original  lesion  which  gave  rise  to  the  palsy.  In-  all  these 
cases  the  epilepsy  is  practically  a  focal  epilepsy,  although 
its  clinical  manifestations  are  not  often  of  the  strictly  Jack- 
sonian  order.  It  was  quite  natural  that  Horsley's  sugges- 
tions with  regard  to  the  proper  treatment  of  focal  epilepsy 
should  be  applied  to  these  forms  of  epilepsy  with  infantile 
palsies. 

Horsley  himself  has  reported  upon  two  such  patients,  and  in  both  of  these 
a  cystic  condition  was  found.  Freud  reports  a  case  of  Oppenheim's  in  which 
a  meningeal  cyst  over  an  arm  centre  was  evacuated,  but  the  brain  itself  was 
not  operated  on  by  the  surgeon  ;  the  athetosis  and  epilepsy  in  these  subjects 
were  considerably  diminished.  The  author  has  had  several  cases  of  cerebral 
palsy  with  epilepsy  operated  upon.  The  first  case,  a  boy,  six  years  of  age,  had 
acquired  right  hemiplegia  at  the  age  of  five,  and  soon  thereafter  developed 
convulsions,  which  invariably  began  in  the  right  hand.  This  right  hand  also 
exhibited  athetoid  movements.  Dr.  Gerster  trephined  over  the  motor  centre 
for  the  right  arm,  and  exposed  the  greater  part  of  the  arm  and  leg  centre  in 
the  left  half  of  the  brain.  The  boy  did  well  for  three  months  after  the  op- 
eration ;  his  attacks  disappeared  altogether,  but  the  ultimate  result  cannot  be 
stated,  as  I  lost  sight  of  the  patient. 

Another  case  was  that  of  a  girl,  sixteen  years  of  age,  with  right  hemiplegia 
and  epilepsy.  Wyeth  performed  the  first  operation  on  this  patient,  exposing 
the  dura  over  the  entire  motor  area,  and  a  very  large  portion  of  the  cortex 
was  inspected  after  the  dura  had  been  incised.  The  exposed  cortical  tissue 
was  tested  by  the  faradic  current,  and  responded  normally  to  mild  currents  ; 
as  the  cortex  seemed  to  be  normal  I  did  not  urge  its  excision.  I  regretted  at 
that  time  not  having  urged  the  complete  excision  of  the  arm  centre.  I  do 
not,  however,  consider  Horsley's  reason  for  removing  such  centres  entirely 
valid,  since  secondary  changes,  which  have  been  established,  seem,  after  all,  to 
be  the  main  source  of  irritation,  and  if  one  focus  of  disease  be  removed  I  am 
confident  that  other  parts  of  the  hemispheres  will  soon  attain  to  the  dignity 
of  epileptogenous  centres.  It  is,  furthermore,  well  established  at  the  present 
day  that  convulsive  seizures  may  result  from  irritation  of  almost  any  part  of 
the  cortex.  In  this  case  there  was  very  little  improvement  excepting  that  the 
epileptic  attacks  were  transferred  after  the  operation  to  the  other  half  of  the 
body,  whose  centres  had  not  yet  been  exposed.  I  have  had  the  other  half 
of  the  brain  exposed  more  recently  by  Dr.  Gerster,  but  for  reasons  which  I 
have  suggested  did  not  urge  the  excision  of  any  of  these  parts,  and  I  fear 
that  the  girl  will  remain  an  incurable  epileptic. 

A  third  case  was  one  operated  on  by  Dr.  Wyeth.  The  patient  was  a 
man,  thirty-two  years  of  age,  who  had  had  an  apopletic  attack  at  the  age  of 
two  years.  As  a  result  of  this  he  exhibited  a  left  hemiplegia  with  considerable 
atrophy,  with  contractures  of  the  left  upper  extremities  and  athetoid  move- 
ments of  the  left  hand  ;  furthermore,  pes  equinus  of  the  left  side  with  a 


INFANTILE    CEREBRAL   PALSIES.  46 1 

tendency  to  equino  varus.  I  made  the  diagnosis  of  an  old  infantile  spastic 
hemiplegia,  probably  due  to  meningeal  hemorrhage  over  the  arm  centre  in 
the  right  hemisphere.  Dr.  Wyeth  exposed  this  centre  according  to  my  di- 
rections, and  at  this  point  was  found  a  distinct  hemorrhagic  discoloration  of 
the  pia,  with  the  development  of  adhesions  between  the  pia  and  the  cortex. 
I  advised  that  these  adhesions  be  incised,  but  did  not  urge  removal  of  the 
cortical  tissue,  though  in  this  case  I  am  free  to  say,  since  the  arm  was  al- 
ready paralyzed  and  practically  useless,  a  very  liberal  excision  of  this  arm 
centre  might  well  have  been  practised.  The  man  recovered  well  from  the 
operation,  and  his  convulsive  seizures  have  been  very  much  diminished  dur- 
ing a  period  of  nearly  three  years.  In  October,  1894,  Dr.  Gerster  operated 
upon  a  fourth  case  (infantile  hemiplegia  with  epilepsy)  in  a  boy,  aged  twelve ; 
the  epileptic  fit  began  in  the  left  (paralyzed)  arm,  and  then  became  general. 
The  greater  portion  of  the  arm  centre  was  excised,  showing  the  effects  of  old 
hemorrhage  (Fig.  145)  ;  the  attacks  have  not  been  sensibly  diminished.  Sim- 
ilar cases  have  been  reported  by  Weir,  Keen,  Roswell  Park,  Angell,  and  Starr. 
In  Weir's  case  a  large  cyst  was  found,  which  was  carefully  drained,  but  the 
child,  four  years  of  age,  died  five  hours  after  the  operation.  In  one  of  Keen's 
cases  the  child  died  twenty  minutes  after  the  operation,  and  the  brain  exhib- 
ited a  very  considerable  atrophy  of  the  entire  left  hemisphere.  The  greater 
danger  of  all  these  operations  in  young  children  should  be  taken  into  ac- 
count, and  for  this  reason  I  now  hesitate  to  have  the  operation  performed 
upon  any  child  under  the  age  of  four  years.  Wildermuth  has  reported  two 
cases.  In  the  one  case  he  removed  atrophic  cortical  tissue,  and  evacuated  a 
cyst  underlying  it.  Three  years  after  the  operation  the  patient  was  said  to 
have  been  improved.  In  another  case  he  also  performed  excision  of  an 
atrophic  portion  of  the  cortex,  and  the  attacks  were  said  to  have  been  in- 
hibited up  to  ten  months  after  the  operation. 

Dr.  Angell,  of  Rochester,  has  reported  upon  a  case  of  infantile  hemiple- 
gia (birth  case)  with  imbecility  and  epilepsy.  A  simple  craniotomy  was 
done,  exposing  a  cyst  in  front  of  and  above  the  ascending  frontal  region  of  the 
left  side  ;  a  slight  improvement  followed.  If  a  cyst  is  as  clearly  limited  as  in 
this  case,  removal  of  the  cyst,  or  at  least  drainage  of  the  same,  would  have 
been  a  more  rational  and  radical  operation  ;  the  improvement  following  upon 
craniotomy  can  be  explained  as  the  result  of  a  release  of  pressure.* 

The  results  of  the  last  few  years  have  been  much  more  encouraging  (cases 
of  Starr,  Chipault,  Krause,  Oppenheim,  Sonnenburg  and  my  own) ;  if  all  the 
symptoms  of  a  case  point  clearly  to  an  initial  focus  of  disease,  surgical  pro- 
cedures are  quite  in  order.  As  cysts  are  extremely  frequent  in  these  infantile 
cerebral  palsies,  the  evacuation  and  removal  of  such  cysts  will  often  be  fol- 
lowed by  considerable  relief.  I  believe  that  it  is  due  to  the  frequency  of  such 
cysts  that  relief  follows  so  many  operations  for  epilepsy. 

If  surgical  procedures  are  unavailing,  the  ordinary  anti- 

*  While  this  edition  was  passing  through  the  press,  Cushing  reported  the  success- 
ful removal  of  large  meningeal  clots  a  few  days  after  birth!  "The  procedure  itself, 
though  delicate  in  performance,  need  not  entail  serious  consequences." 


462  THE  NERVOUS  DISEASES   OF   CHILDREN. 

epileptic  measures  may  be  employed.  Bromides  and  chlo- 
ral should  be  given  in  moderate  doses,  but  there  is  little 
use  in  pushing  these  drugs  to  the  extreme,  for  we  cannot 
expect  to  cure  an  epilepsy  that  is  dependent  upon  an  organic 
brain  lesion. 

It  will  be  best  in  this  connection  to  study  a  disease  which 
has  evident  relations  to  other  family  affections  (see  Chapter 
XXI).  Although  it  is  a  cerebro-spinal  disorder,  the  author's 
studies  on  this  disease  were  made  largely  in  connection 
with  the  study  of  the  infantile  cerebral  palsies. 

AMAUROTIC   FAMILY  IDIOCY. 

This  name  was  given  by  the  author  to  a  disease  not  altogether  rare  * 
affecting  several  members  of  one  family,  and  characterized  by  an  impairment, 
of  all  the  mental  functions,  by  a  progressive  weakness  of  some  or  of  all  the 
muscles  of  the  body,  and  by  a  defect  in  vision  (associated  with  changes  in 
the  macula  lutea  and  subsequent  optic  nerve  atrophy),  terminating  in  com- 
plete blindness.  The  disease  is  fatal,  the  children  dying,  as  a  rule,  in  a  con- 
dition of  complete  marasmus  before  the  end  of  the  second  year  of  life. 

History  of  the  Disease. — In  1881  Waren  Tay  described  "symmet- 
rical changes  in  the  region  of  the  yellow  spot  in  each  eye  of  an  infant."  He 
had  noted  the  fact  that  the  child's  cerebral  development  was  slow,  and  also 
that  the  optic  discs  were  apparently  healthy  at  first,  but  that,  in  the  region  of 
the  yellow  spot,  there  was  a  conspicuous,  tolerably  large  white  spot  more  or 
less  circular  in  outline,  and  showing  at  its  centre  a  brownish-red,  fairly  circu- 
lar spot  contrasting  strongly  with  the  white  spot  surrounding  it.  Tay  likened 
this  "  cherry-red  spot  "  to  the  appearances  which  are  well  known  in  cases  of 
embolism  of  the  central  artery  of  the  retina,  and  believed  the  changes  to  be 
"  possibly  congenital."  Subsequently  he  noticed  a  gradually  developing  atro- 
phy of  the  optic  discs,  while  the  changes  in  the  macula  lutea  remained  the 
same  as  before.  Tay  also  reported  that  three  similar  cases  had  occurred  in 
this  family.  These  same  ophthalmoscopic  findings  had  been  noted  by  a 
number  of  oculists  in  Europe  and  in  America. 

So  long  ago  as  1887,  without  any  knowledge  of  the  cases  described  by  the 
oculists,  the  author  reported  what  appeared  to  be  a  peculiar  form  of  idiocy 
associated  with  blindness.  Some  years  later  I  became  aware  of  the  family 
character  of  this  affection,  and  it  was  then  shown  by  Kingdon,  of  Nottingham, 
that  the  cases  reported  by  the  oculists  and  those  reported  by  me  were  iden- 
tical. But  it  was  not  until  the  year  1898,  after  a  much  larger  experience 
with  this  category  of  cases,  that  I  was  convinced  that  this  condition  deserved 
to  be  considered  as  a  clinical  entity,  and  in  that  year  I  proposed  the  name 
Amaurotic  Family  Idiocy,  which  has  been  universally  adopted. 

*  In  1904  Heveroch  had  collected  the  records  of  86  cases,  of  which  44  occurred  in 
America. 


AMAUROTIC   FAMILY  IDIOCY. 


4^3 


Risien  Fussell  has  been  the  only  one  to  use  the  designation  "Infantile 
Cerebral  Degene"ralion.:'    The  name  is  wholly  inadequate,  and  in  view  of  our 
p-esent  knowledge  of  the  pathology  of  the  disease,  does  not  do  justice  even 
to  the  morbid  anatomy  of  Am- 
aurotic Idiocy.   The  symptoms 
as  described  by  me  have  been 
endorsed    by    all    subsequent 
writers.     Among  these  I  wish 
especially  to  mention  Peterson, 
Hirsch,  Koplik,  Kingdon,  Rus- 
sell, Falkenheim,  Higier,  Schaf- 
fer,  Fry,  Spiller,  McKee,  Mary 
Buchanan,  and  Shumway. 

Symptomatology.  —  The 
children  are  born  healthy  (sev- 
eral of  them  have  been  unusu- 
ally robust)  and  do  well  until 
the  age  of  four,  five  or  six 
months.  At  about  that  time 
they  become  listless,  apathetic, 
move  their  limbs  very  little, 
and  show  the  first  signs  of  im- 
paired vision.  The  child  is 
unable  to  hold  its  head  up 
straight  or  to  sit  up,  and  as 
time  goes  on,  instead  of  gain- 
ing strength  in  the  use  of 
its  muscles,  its  limbs  become 
limp,  in  some  instances  spastic 
and  more  or  less  paralyzed. 
The  reflexes  may  remain  nor- 
mal, at  times  they  may  be  a 
trifle  subnormal,  and  in  the 
spastic  cases  they  become  ex- 
aggerated. An  unusual  sensitiveness  to  sound  (hyperacusis)  is  observed  at 
a  very  early  date,  so  that  the  child  is  startled  by  the  slightest  noise,  by  a 
clapping  of  the  hands,  etc.  Oppenheim  speaks  of  this  symptom  as  an  in- 
creased motor  acoustic  reflex.  Convulsions  may  occur,  but  are  not  an  integral 
feature  of  the  disease.  After  the  lapse  of  a  few  months  the  mental  impair- 
ment is  distinctly  increased  and  blindness  becomes  absolute,  and  the  child 
lapses  into  a  condition  of  extreme  marasmus,  in  which  it  dies,  as  a  rule,  be- 
fore the  end  of  the  second  year  (Fig.  1 15a). 

We  may  sum  up  the  chief  symptoms  as  follows:  (1)  A  mental  impair- 
ment observed  during  the  earlier  months  of  life  and  leading  to  absolute  idiocy. 
(2)  A  paresis  or  paralysis  of  the  greater  part  of  the  body  which  may  be  either 
flaccid  or  spastic.  (3)  The  reflexes  may  be  normal,  deficient,  or  increased. 
(4)  A  diminution  of  vision  terminating  in  absolute  blindness  (cherry-red  spot 


Fig.  115. — Case  of  Amaurotic  Family  Idiocy,  14 
months  old.  Patient  of  Dr.  Cotton,  of  Chicago, 
showing  well-nourished  condition  of  child  in 
earlier  stage  of  disease. 


464 


THE  NERVOUS  DISEASES   OF  CHILDREN. 


in  the  region  of  the  macula  lutea  with  subsequent  opt'c  nerve  atrophy). 
(5)  Marasmus,  and  a  fatal  termination,  as  a  rule,  at  ab^A1  me  age  of  two 
years.  (6)  Unusual  sensitiveness  to  sound  (hyperacusis).  (7)  The  appear- 
ance of  the  affection  in  several  members  of  the  same  family. 

In  some  of  the  children  I  have  noted  nystagmus  and  strabismus.  A 
few  other  symptoms  in  addition  to  those  described  above  have  been  men- 
tioned by  Falkenheim.  These  are  "  explosive  laughter  "  and  "  disturbances 
of  deglutition."  The  former  I  have  not  observed,  but  it  is  very  probable  that 
it  may  occur  in  some  of  the  cases,  and  the  latter  may  be  taken  to  be  an 
accompaniment  of  the  great  mental  and  physical  deterioration  ;  and  the  occur- 
rence of  such  bulbar  symptoms  need  not 
astonish  one,  in  view  of  our  present 
knowledge  of  the  anatomy  of  the  disease. 
The  ocular  symptoms  constitute  a 
most  striking  feature  of  the  disease.  In 
every  instance  in  which  the  opportunity 
for  ophthalmoscopic  examination  has 
been  given,  this  peculiar  condition  was 
found  present,  but  I  wish  to  state  dis- 
tinctly that  the  disease  can  be  diagnosti- 
cated, as  has  been  done,  before  the  oc- 
currence of  the  typical  changes  in  the 
region  of  the  macula  lutea.  In  Hig- 
ier's  case  optic  nerve  atrophy  was  much 
more  pronounced  than  the  changes  in 
the  macula  lutea.  All  writers  on  the 
subject  have  fully  appreciated  the  char- 
acteristic symptoms  of  Amaurotic  Idiocy, 
but  the  importance  of  the  rapidly  devel- 
oping marasmus  has  not  been  sufficiently 
insisted  upon  by  many  of  them.  I  hold 
it  to  be  one  of  the  important  symptoms 
of  the  condition. 

Etiology. — The  causes  underlying 
this  disease  are  still  somewhat  obscure. 
Blood  relationship  between  the  parents 
has  been  noted  in  some  instances  and  a 
marked  psycho-neurotic  taint  in  others. 
In  a  few  instances,  the  mother  has  sus- 
tained  some  injury  during  pregnancy. 
The  family  predisposition  is  made  evi- 
dent by  the  fact  that  twenty-eight  cases 
which  came  under  my  notice  have  oc- 
curred in  fifteen  families.    Carter  was  the 
first  to  call  attention  to  the  fact  that  all  cases  reported  have  occurred  among 
Hebrews,  and  up  to  the  present  time  not  a  single  entirely  satisfactory  case 
has  been  reported  as  occurring  among  other  races.     It  is  truly  astounding 


Fig.  115a. — Child  in  Last  Stages  of 
Amaurotic  Family  Idiocy,  showing 
extreme  emaciation  and  (unusual) 
contractures.     Author's  case. 


AMAUROTIC  FAMILY  IDIOCY. 


465 


that  this  disease  should  be  thus  limited,  because  other  diseases  to  which  it  is 
more  or  less  closely  allied  have  been  observed  among  all  races  and  all 
nationalities. 

It  is  well  to  state  explicitly  that  this  is  not  a  syphilitic  disease,  and  this 
point  should  be  insisted  upon,  because  hereditary  optic  nerve  atrophy  occur- 
ring later  in  life  has  been  shown  to  be  due  frequently  to  hereditary  syphilis, 
and  there  are  other  family  affections  associated  with  dementia  which  are  due 
to  this  same  cause. 

Diagnosis. — There  can  be  little  difficulty  in  diagnosticating  this  form  of 
disease  if  the  symptoms  as  enumerated  above  are  kept  in  mind.  Hereditary 
spastic  diplegia,  which  sometimes  occurs  in  families,  may  give  rise  to  some 
confusion,  but  in  this  disease  the  entire  absence  of  visual  symptoms,  the  lark 
of  mental  impairment,  the  marked  spasticity  of  all  limbs,  and  the  fact  that  the 
children  show  no  tendency  to  marasmus  will  help  to  bring  out  the  true  diag- 
nosis. Hereditary  optic  atrophy  may  also  be  confounded  with  Amaurotic 
Idiocy,  but  the  tormer  only  occurs  in  older  persons.  Several  times  the  author 
has  been  asked  to  see  cases  which  were  supposed  to  be  instances  of  Amau- 
rotic Family  Idiocy  which  presented  the  symptoms  due  to  a  preceding  men-(}> 
ingitis ;  and  on  the  other  hand,  the  author  is  also  aware  that  in  not  a  few 
instances  the  diagnosis  of  meningitis  has  been  made  when  the  case  was  a 
very  typical  one  of  Amaurotic  Family  Idiocy.  Rayner  D.  Batten  has  re- 
ported symmetrical  disease  of  the  macula  lutea  in  two  brothers  beginning  at 
the  age  of  14  years,  and  F.  E.  Batten  describes  "  cerebral  degeneration  " 
with  symmetrical  changes  in  the  macula  lutea  in  two  members  of  a  family. 
These  cases  are,  however,  very  distinct  from  the  type  here  described.  In 
Rayner  D.  Batten's  cases  there  was  hereditary  syphilis ;  yet  it  is  of  interest  to 
note  the  family  occurrence  of  ocular  changes  in  and  around  the  macula  lutea. 


J 


® 


Pathological  Anatomy.— In  three  brains  which  the  author  has  had 
an  opportunity  of  examining  there  were  peculiar  abnormalities  of  fissura- 


466 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


tion  (confluence  of  the  fissure  of  Rolando  and  fissure  of  Sylvius  and  in  two 
instances  exposure  of  the  island  of  Reil).  Such  abnormalities  are  generally 
conceded  to  be  a  sign  of  deficient  cerebral  development  (Fig.  116).  In  the 
earlier  publications  on  this  subject  I  spoke  of  the  morbid  condition  as  an 
Agenesis  corticalis,  a  defect  in  the  development  of  the  highest  nerve  elements 
of  the  cortex.  There  was  also  some  degeneration  of  the  white  fibres  in  the 
hemispheres  and  of  the  pyramidal  tracts  in  the  spinal  cord.  This  degeneration 
of  the  white  fibres  could  be  traced  through  the  course  of  the  pyramidal  tracts 
in  the  internal  capsule,  crusta,  pons  and  medulla.     But  more  striking  than 


Fig.   117. — Section  through  XII  nucleus  in  medulla  oblongata,  showing  advanced 
changes  in  ganglion  cells.     Hematoxylin-eosin  stain. 


this  change  in  the  white  fibres  is  the  change  in  the  gray  matter  not  only  of 
the  cortex,  but  throughout  the  entire  central  nervous  system.  The  changes 
are  found  to  be  the  same  in  the  cortex  of  the  brain,  in  the  cranial  nerve  nu- 
clei, and  in  the  anterior  and  posterior  gray  matter  from  the  cervical  to  the 
lumbar  and  sacral  segments  of  the  cord.  Even  the  spinal  ganglia  showed 
similar  changes.  Our  knowledge  of  the  spinal  changes  we  owe  chiefly  to 
Dr.  Hirsch,  whose  findings  I  have  been  able  to  corroborate  in  a  case  exam- 
ined by  me  only  a  few  years  ago.  There  can  no  longer  be  any  doubt  that 
the  disease  is  due  to  a  degeneration  of  the  ganglion  cells  throughout  the  en- 
tire central  nervous  system.     In  the  cortex,  as  well  as  in  the  gray  matter  of 


AMAUROTIC  FAMILY  IDIOCY. 


467 


the  cord,  there  is  scarcely  a  normal  ganglion  cell  to  be  seen.     As  will  be 
noted  from  the   illustrations   herewith   presented  (see  Figs.   117-119;,  the 


Fig.  118. — Section  through  cervical  enlargement,  showing  cell  changes  in  anterior 
gray  matter.     (Van  Gieson  stain. ) 

cell-body  is  completely  altered ;  the  entire  cell  protoplasm  has  become  disin- 
tegrated, leaving  a  more  or  less  homogeneous  mass;  the  nucleus  has  been 


Fig.   119.— Section  through  a  lumbar  ganglion.     Nissl  stain  showing  marked  cell 

changes. 

shifted  commonly  to  some  part  of  the  periphery  of  the  cell,  and  instead  of 
being  distinctly  differentiated  from  the  rest  of  the  cell-body,  it  generally 


468  THE  NERVOUS  DISEASES   OF   CHILDRE-N. 

shades  off  by  degrees  into  the  disintegrated  structure  of  the  cell.  The  nu- 
cleus is  often  wanting  entirely,  and  in  some  instances  the  whole  nucleus  ap- 
pears to  have  dropped  out.  The  disintegration  of  the  cell-body  is  so  complete 
that  in  many  specimens  the  contour  alone  enables  one  to  infer  that  these 
round  bodies  are  the  remnants  of  ganglionic  cells.  These  metamorphosed 
ganglion  cells  are  frequently  surrounded  by  distinctly  pericellular  spaces,  and 
are  relatively  and  absolutely  so  much  enlarged  that  they  give  a  very  striking 
appearance  to  the  cross-section. 

Holden,  as  well  as  Shumway  and  Mary  Buchanan,  have  made  careful  his- 
tological examination  of  the  eyes  in  Amaurotic  Family  Idiocy.  All  these 
writers  are  agreed  that  the  essential  changes  in  the  eyes  are  "a  degeneration 
of  the  ganglion-cells  of  the  retina  and  of  the  nerve-fibres  of  the  optic  nerves 
and  tracts,  which  are  genetically  a  portion  of  the  central  nervous  system." 
They  also  believe  that  the  white  area  in  the  fundus  is  the  result  "  of  the 
swollen  and  degenerated  ganglion-cells  which  are  present  in  much  greater 
numbers  in  the  macula  region  than  elsewhere,"  and  do  not  consider  this  white 
area  to  be  due  to  an  oedema  of  the  tissue  as  was  first  supposed. 

Hirsch,  Spiller,  Shaffer,  of  Budapest,  and  the  author  have  proved  that  Am- 
aurotic Family  Idiocy  is  due  to  a  degeneration  of  the  entire  gray  matter  of  the 
brain  and  spinal  cord.  The  degeneration  of  the  white  fibres  of  the  anterior 
and  lateral  pyramidal  tracts  is  in  all  probability  secondary,  but  not  nearly  so 
marked  as  one  would  expect  with  such  advanced  disease  of  the  ganglionic 
elements.  It  is  probable  that  the  relative  involvement  of  the  gray  and  white 
matter  may  vary  much  in  different  subjects  affected  with  this  disease. 

Hirsch  and  some  others  have  supposed  that  Amaurotic  Family  Idiocy  was 
an  acquired  disease;  that  the  morbid  changes  in  the  cells  were  of  a  distinctly 
degenerative  character,  and  that  these  changes  were  similar  to  those  produced 
by  toxic  agents  of  various  sorts.  Hirsch  went  to  the  extent  of  claiming  that 
the  toxic  agent  might  possibly  be  conveyed  to  the  child  through  the  mother's 
milk.  It  is  difficult  to  explain  any  family  disease  on  the  supposition  that  it  is 
of  toxic  origin,  and  in  this  instance  the  author  must  repudiate  this  view  for  the 
simple  reason  that  several  of  his  patients  have  not  been  nursed  by  their  own 
mother;  and  it  is  very  evident  that,  although  the  symptoms  of  the  disease  are 
not  always  noticed  before  the  age  of  three  or  four  months,  the  development 
of  the  disease  is  due  to  conditions  antedating  the  time  of  birth.  The  author 
still  firmly  believes  that  Amauratic  Family  Idiocy  is  due  to  an  arrest  of  de- 
velopment, and  that  this  arrest  of  development  is  followed  by  a  degeneration. 
Accepting  Gowers'  and  Edinger's  views  of  the  inherent  or  deficient  capacity 
for  normal  development,  we  may  simply  argue  that  children  who  are  afflicted 
with  Amaurotic  Family  Idiocy  have  a  very  limited  capacity  for  normal  de- 
velopment ;  that  their  central  nervous  system  is  not  equal  to  the  functions  that 
they  are  expected  to  perform  for  more  than  the  first  three  or  four  months  of 
life,  and  from  that  time  on  a  rapid  deterioration  of  all  functions  is  established. 

Prognosis. — The  disease  is  invariably  fatal ;  between  the  age  of  twelve 
and  eighteen  months  the  children,  even  though  they  have  been  in  tolerably 
robust  health  at  birth,  pass  into  a  condition  of  extreme  marasmus.     Only  one 


HEREDITARY  SPASTIC  PALSIES.  tfxj 

case  in  which  the  diagnosis  of  Amaurotic  Family  Idiocy  could  be  accepted 
has  lived  beyond  the  age  of  two  years. 

Treatment. — No  form  of  treatment  has  been  of  any  avail.  It  is  very 
positive  that  the  disease  is  not  due  to  hereditary  syphilis,  and  for  that  reason 
even  the  ordinary  iodide  treatment  cannot  be  urged.  In  several  families  in 
which  children  with  this  disease  have  been  observed,  others  have  been  born 
and  have  remained  entirely  healthy.  No  further  warning  should  therefore  be 
given  to  parents  of  such  children  ;  let  them,  however,  exercise  special  care 
with  reference  to  the  physical  development  and  early  training  of  their  off- 
spring. 

Freud  has  reported  several  interesting  cases  under  the  title  of  "  Family 
Forms  of  Cerebral  Diplegia."  Two  of  the  children  were  bright  at  the  ages 
of  six  and  five  years  respectively,  but  they  presented  from  earliest  childhood 
the  following  symptoms  :  Nystagmus,  atrophy  of  the  optic  nerve,  convergent 
strabismus,  awkwardness  in  speech  and  in  the  use  of  the  arms ;  tremor  of 
the  arms  and  a  spastic  paraplegia  of  the  lower  extremities  ;  no  convulsions. 
In  these  boys  the  disease  is  evidently  present  in  its  lightest  and  most  favor- 
able form.  The  improvement  in  vision  as  the  one  child  grew  older  seems 
evidence  of  this,  but  the  resemblance  of  these  forms  to  those  described  by 
me  is  shown  by  a  third  child  that  died  at  the  age  of  ten  months ;  it  was 
paralyzed  from  birth,  and  idiotic  ;  whether  it  was  blind  also  is  not  stated.  The 
father  thought  the  child  had  died  of  rickets  (more  probably  of  marasmus). 

Pelizaeus  has  described  a  family  disease  of  which  the  chief  symptoms 
were :  Nystagmus,  mental  imbecility,  disturbances  of  speech  (Bradylalia), 
awkwardness  of  hands,  spastic  paraplegia  of  the  lower  extremities.  The 
disease  attacked  male  members  of  several  generations,  but  was  transmitted 
through  healthy  mothers.  All  of  those  who  were  attacked  showed  some 
symptoms  in  childhood,  and  several  died  young.  Pelizaeus  was  inclined  to 
the  diagnosis  of  multiple  sclerosis ;  but  his  cases  evidently  constitute  a  late 
form  of  hereditary  spastic  paralysis. 


CHAPTER   XXVI. 

TUMORS   OF   THE   BRAIN   AND    ITS   MENINGES. 

New-growths  within  the  skull  are  very  common  in 
childhood.  There  is  no  period  of  early  life  that  is  entirely 
exempt  from  them.  Careful  studies  of  cerebral  tumors 
have  been  made  by  Steffen,  Bernhardt,  Starr,  Knapp,  Bruns, 
and  Oppenheim.  The  following  tables  are  taken  from  an 
article  by  Peterson : 

Table  I. 
Form  of  Tumor.  No.  of  Cases. 

Tubercle 166 

Glioma 42 

Sarcoma 37 

Cyst 35 

Carcinoma 1 1 

Gliosarcoma 5 

Angiosarcoma i 

Myxosarcoma i 

Papillary  epithelioma i 

Gumma i 

Not  stated 35 

Total 335 

Table  II. 

Site  of  Tumor.                                                                                        No.  of  Cases. 

Cerebellum 105 

Pons  Varolii 42 

Centrum  ovale 41 

Basal  ganglia  and  lateral  ventricles 30 

Corpora  quadrigemina  and  crura  cerebri 25 

Cortex  cerebri 23 

Medulla  oblongata 7 

Fourth  ventricle 6 

Base  of  brain 8 

Total 287 


TUMORS   OF  THE   BRAIN  AND  ITS  MENINGES.         471 

From  these  tables  we  can  infer,  first  of  all,  that  tubercle  is  much  more 
frequent  than  any  other  form  of  intra-cranial  neoplasm,  very  nearly  one-half 
of  all  the  cases  being  of  this  character.  Glioma  and  sarcoma  are  the  next 
most  common  forms,  and  as  the  cerebral  tissue  in  earlier  years  is  subject  to 
proliferation,  the  frequent  occurrence  of  the  former  need  not  be  a  matter  of 
surprise.  Cysts  differ  from  tumor  in  this  respect  that  they  are  secondary 
products  of  some  preceding  morbid  process,  very  often  resulting  from  an  old 
hemorrhage,  or  from  embolic  softening.  The  contents  of  a  cyst  may  remain 
stationary  for  a  very  long  time,  and  under  such  circumstances  the  symptoms 
will  not  be  very  like  those  of  a  growing  tumor.  In  other  instances  the  con- 
tents of  the  cyst  suddenly  increase  and  all  the  symptoms  may  be  those  of  a 
rapidly  increasing  neoplasm. 

As  for  the  location  of  tumors  in  general,  it  will  be  seen  that  a  larger 
number  are  found  in  the  cerebellum  than  in  ayy  other  part  of  the  brain. 
The  pons  and  the  centrum  ovale  are  next  in  order  of  frequency,  then  come 
the  basal  ganglia,  and  last  the  cortex  of  the  brain,  and  the  region  of  the  cor- 
pora quadrigemina.  The  medulla  oblongata  and  the  base  of  the  brain  are 
rarely  the  seat  of  tumor. 

Etiology. — The  actual  cause  of  cerebral  tumors  in 
children  is  a  matter  of  considerable  speculation.  It  is  only 
with  regard  to  gliomata  that  we  can  state  that  the  natural 
tendency  of  the  infantile  brain  to  proliferation  of  its  tissue 
upon  the  slightest  provocation  accounts  for  the  frequent 
occurrence  of  this  special  form  of  neoplasm.  Tubercle, 
sarcoma,  and  carcinoma  of  the  brain,  are  always  secondary 
to  deposits  of  a  similar  character  in  other  parts  of  the  body. 
Carcinoma  is  rare  in  children,  and  carcinomatous  deposits 
in  the  brain  are  correspondingly  infrequent,  but  under  excep- 
tional conditions  these  may  occur  early  in  life.  A  few  cases 
have  been  observed  which  were  due  to  an  invasion  of  the 
growth  from  the  orbit.  The  exact  relation  of  external  in- 
juries to  new-growths  has  been  often  in  dispute.  It  is  more 
than  probable  that  the  accident  to  which  the  tumor  is  re- 
ferred is,  as  a  rule,  merely  a  landmark  in  the  child's 
history. 

In  the  fewest  instances  only  can  an  actual  causal  relation  between  the  two 
be  established.  The  hyperaemic  condition  or  the  slight  hemorrhages  which 
occur  at  the  seat  of  injury  may  at  times  be  sufficient  to  become  the  starting- 
point  of  a  latent  morbid  process.  Boys  are  more  liable  to  brain  tumors  than 
girls,  and  those  who  are  not  too  careful  in  utilizing  statistical  evidence  will 
claim  that  this  fact  is  to  be  explained  by  the  greater  frequency  of  injuries  to 
the  boys'  skulls. 


47 2  THE  NERVOUS  DISEASES   OF   CHILDREN. 

Symptoms. — As  in  the  adult,  so  in  the  child,  the  symp. 
toms  of  intra-cranial  tumor  may  be  divided  into  two  distinct 
groups.  The  first  includes  the  general  symptoms  resulting 
from  increased  pressure,  and  the  second  group  includes 
those  due  entirely  to  the  location  of  the  tumor.  It  is  a 
matter  of  course  that  slowly  progressive  tumors  will  exhib- 
it less  symptoms  than  those  which  invade  the  brain  rapidly 
and  soon  attain  to  a  very  considerable  size.  A  tumor  of 
small  size  growing  rapidly,  will  produce  far  more  symp- 
toms than  one  of  huge  size  which  has  taken  years  to  de- 
velop. The  symptoms  will  also  vary  according  to  the 
blood-supply  of  the  tumor,  and  according  to  the  increase 
in  the  amount  of  hydrocephalic  fluid.  The  general  symp- 
toms which  we  must  consider  somewhat  in  detail  are  head- 
ache, nausea,  vomiting,  stupor,  insomnia,  convulsions,  slow 
pulse,  and  double  optic  neuritis. 

Headache  is  in  many  cases  the  most  striking  symptom  of  tumor  of  the 
brain.  The  nearer  the  tumor  is  to  the  cortex,  and  the  more  it  involves  the 
meninges,  the  more  intense  on  the  whole  the  headaches  are  likely  to  be ;  but 
I  have  seen  headaches  as  intense  as  those  of  cortical  tumors  in  cases  in  which 
the  growth  was  in  the  vicinity  of  the  ganglia,  or  even  at  the  base  of  the  brain. 
In  such  patients  the  increase  in  cerebro-spinal  fluid  has  much  to  do,  in  all 
probability,  with  the  development  of  the  headaches.  The  intense  "  boring," 
"  gnawing  "  pain  is  generally  referred  to  that  part  of  the  head  which  is  nearest 
to  the  new-growth  ;  but  there  are  exceptions  to  this  rule  as  to  every  other. 
In  children  whose  fontanelles  are  not  completely  closed  the  tumors  may 
attain  to  a  considerable  size  without  causing  intense  headaches.  These 
headaches  are  often  the  cause  of  sleeplessness,  and  both  the  pain  and  the 
insomnia  are  largely  responsible  for  the  rapid  emaciation  of  the  child.  In 
some  few  instances  insomnia  is  present  without  pain,  and  is  the  result  of 
cerebral  excitement. 

The  association  of  nausea  and  vomiting  with  headaches  in  a  child  should 
lead  to  the  suspicion  of  tumor,  particularly  if  all  these  symptoms  persist  for 
some  considerable  period  of  time.  The  vomiting  is  characterized  by  its  sud- 
denness, and  by  its  projectile  character.  Nausea  may  be  associated  with  it, 
but  in  many  cases  there  is  no  warning  given  of  the  vomiting,  and  it  comes  as 
a  surprise  upon  the  child,  and  often  occurs  entirely  independently  of  meals  ; 
at  other  times  during  meals.  Vertigo  is  not  infrequent  in  cases  of  tumor,  and 
is  very  apt  to  occur  with  every  change  in  position  of  the  head.  It  is  very  fre- 
quent indeed  in  connection  with  tumors  of  the  cerebellum,  or  of  the  pons, 
which  is  explained  by  the  relation  of  these  parts  to  the  auditory  nerve. 

Convulsions  in  children  are  so  common  that  they  alone  need  not  give  rise 
to  the  suspicion  of  tumor,  but  if  associated  with  constant  headaches,  with 


TUMORS    Ofi   THE   BRAIN  AND   ITS  MENINGES.  473 

vomiting,  and  if  all  these  symptoms  are  frequently  repeated,  the  suspicion  may 
be  well  founded.  These  convulsions  are  very  often  general,  but  in  addition 
to  the  general  convulsions,  or  without  the  occurrence  of  such,  localized  con- 
vulsions may  occur  if  the  neoplasm  is  in  some  part  of  the  motor  area.  Such 
localized  convulsions,  associated  with  headaches,  vomiting,  and  with  optic 
neuritis,  point  very  definitely  to  an  intra-cranial  growth. 

Double  optic  neuritis  and  the  complete  optic  atrophy 
following  it  are  important  symptoms  of  intra-cranial  tu- 
mor. It  is  certain  that  there  is  no  symptom  which  if  pres- 
ent, is  more  valuable  than  an  optic  neuritis  ;  but,  on  the 
other  hand,  tumors  may  attain  considerable  size  without 
the  development  of  optic  neuritis.  This  neuritis  is  in  all 
probability  due  to  increase  of  intracranial  pressure.  Other 
theories  which  have  been  proposed  do  not  explain  all  the 
peculiarities  of  optic  neuritis  as  well  as  the  theory  of  in- 
creased pressure  does.  The  fundus  should  be  examined  in 
every  doubtful  case  of  brain  tumor,  for  normal  vision  is 
not  incompatible  with  a  considerable  degree  of  optic  neu- 
ritis, and  changes  in  the  optic  nerve  may  occur,  .and  do  oc- 
cur, with  tumor  in  any  part  of  the  brain  ;  but  they  occur  at 
a  much  earlier  period  and  to  a  much  more  intense  degree 
with  tumors  situated  at  the  base  of  the  brain.  Under  these 
conditions  the  direct  pressure  upon  the  optic  chiasm  and 
the  greater  increase  of  intra-cranial  fluid  at  the  base  may 
account  for  the  earlier  appearance  of  the  symptoms.  The 
optic  neuritis  is  generally  double,  though  it  may  be  more 
distinct  for  a  time  in  one  eye  than  in  another. 

Sudden  variations  in  the  pulse-rate,  and  in  rapidity  of  res- 
piration have  been  observed  ;  Cheyne-Stokes  respiration  I 
have  seen  in  children  suffering  from  cerebellar  tumors  long 
before  the  terminal  stage  had  been  reached. 

The  presence  of  tumor  can  often  be  suspected  from  the 
occurrence  of  these  general  symptoms.  The  signal  or 
localizing  symptoms  are  dependent  upon  the  special  func- 
tions of  the  areas  invaded  by  the  neoplasm.  These  localiz- 
ing symptoms  are  the  best  illustrations  in  man  of  cerebral 
functions,  as  they  have  been  demonstrated  by  experiments 
upon  animals.  The  matter  is  not  quite  as  simple,  however, 
as  it  would  seem,  for  a  neoplasm  causes  both  direct  and  in- 
direct  symptoms.     The  direct  symptoms  are  those  caused 


474  THE  NERVOUS  DISEASES    OF   CHILDREN. 

by  the  actual  loss  of  function  due  to  displacement  of  tissue 
by  the  neoplasm.  The  indirect  symptoms  are  caused  either 
by  the  distant  effects  of  the  pressure  by  the  tumor  upon 
neighboring-  parts,  or  they  may  result  from  the  secondary 
increase  of  intra-cranial  fluid.  At  all  events,  the  symptoms 
first  produced  are  the  most  important  ones,  and  will  be  of 
the  greatest  value  in  determining  the  exact  site  of  the 
lesion. 


The  indirect  symptoms  are  perhaps  more  frequent  in  the  cases  of  cere- 
bellar tumors  than  in  any  other.  Thus  in  this  form  we  may  have  the  gen- 
eral symptoms  of  intra-cranial  neoplasm,  together  with  reeling  and  distinct 
occipital  headaches.  Associated  with  these  we  often  find  a  paralysis  of  the 
rectus  externus  supplied  by  the  sixth  nerve.  Spinal  symptoms  may  occur 
with  cerebellar  and  other  intra-cranial  growths.  Batten  and  Collier,  Anton, 
Pick,  and  a  number  of  German  writers  have  referred  to  the  frequent  involve- 
ment of  spinal  cord  structures  (the  posterior  roots  and  posterior  columns), 
evidently  due  to  the  increased  pressure  of  the  cerebro-spinal  fluid. 

Macewen's  Symptom. — "The  elicitation  of  a  differential  cranial  per- 
cussion-note as  an  aid  to  cerebral  diagnosis  in  certain  gross  changes  of  the 
intra-cranial  contents,  especially  in  children,"  has  been  practised  by  Macewen 
for  many  years  and  described  in  his  monograph  on  "  Infective  Diseases  of  the 
Brain  and  Spinal  Cord."  For  the  present,  the  symptom  is  most  valuable  in 
the  case  of  abscesses  and  of  over-distended  lateral  ventricles.  In  cases  of 
tumor  near  the  surface  of  the  brain  the  percussion-note  will  prove  of  service. 
I  have  noted  a  dulness  in  several  cases  of  tumor  of  the  brain  (one  case  in 
the  adult  and  two  in  children) ;  if  the  lateral  ventricles  are  over-distended 
the  resonance  is  greatly  increased.  But  distention  of  the  ventricles  may  be 
secondary  to  tumor,  and  in  this  way  increased  resonance  of  the  percussion- 
note  may  be  significant.  The  percussion-note  of  normal  crania  seems  to  vary 
much  ;  it  will  be  best,  therefore,  to  use  this  symptom  very  cautiously.  The 
note  can  be  elicited  best  by  tapping  the  skull  lightly  with  the  finger  or 
percussion-hammer,  and  receiving  the  note  through  a  stethoscope  placed 
upon  the  pterion,  or  upon  the  middle  of  the  forehead. 

Tumors  of  the  Cortex. — It  is  not  an  easy  matter  to  distinguish  be- 
tween the  purely  cortical  tumors  and  those  situated  in  the  subjacent  white 
matter.  Some  claim  that  it  is  impossible  to  differentiate  between  cortical 
and  subcortical  tumors,  but  this  seems  to  me  to  be  rather  overstating  the 
facts.  While  the  localizing  symptoms  may  be  exactly  the  same  in  both 
these  classes  of  tumors,  the  order  of  development  of  the  symptoms  will 
be  different,  and  may  give  some  indication  of  the  exact  site  of  the  tumor. 
In  subcortical  tumors  the  march  of  the  symptoms  is  generally  such  that 
the  approach  of  the  lesion  toward  the  cortex  is  marked  by  a  special  series 
of  symptoms.  Taking,  for  example,  the  tumors  in  the  motor  area,  those 
in  or  near  the  gray  matter,  however  small,  are  apt  to  give  rise  to  occa- 


TUMORS    OE   THE   BRAIN  AND   ITS   MENINGES.  475 

sional  convulsive  seizures  from  the  very  start ;  whereas,  in  the  tumors  which 
begin  in  the  subjacent  white  matter,  the  paralytic  symptoms  may  appear  at 
a  very  early  day  and  may  exist  for  some  time  before  any  symptoms  of  corti- 
cal irritation  arise.  Thus,  in  a  case  under  my  observation,  the  patient  had 
gradually  developed  paralysis  of  the  arm,  of  the  face,  and  slight  paresis  of 
the  leg  on  one  side  of  the  body.  He  had  indistinct  headaches  and  optic 
neuritis,  and  yet,  during  the  first  three  months  after  the  onset  of  these  symp- 
toms, not  a  single  convulsive  twitching  occurred.  Since  that  period  epilep- 
toid  seizures  have  set  in,  and  the  occurrence  has  proved  that  the  tumor  has 
finally  invaded  the  cortical  region.  The  invasion  of  the  cortex,  moreover,  is 
more  likely  to  lead  to  repeated  convulsive  seizures  and  to  increased  head- 
aches from  the  more  direct  involvement  of  the  meningeal  coverings;  the 
nearer  the  surface  and  the  nearer  the  meninges  the  more  intense  these  head- 
aches become.*  Certain  classes  of  tumors,  moreover,  are  more  apt  to  begin 
in  the  cortical  tissue  than  in  the  subcortical  white  substance  ;  thus  tubercles, 
gliomata,  and  gummata,  above  all,  are  almost  certain  to  begin  near  the  sur- 
face, while  sarcomata  and  cysts  are  as  often  subcortical  as  cortical  in  origin. 

Tumors  of  the  Frontal  Lobe.— Since  the  frontal  lobe  has  no  dis- 
tinct and  special  function,  tumors  in  this  region  are  often  developed  without 
localizing  symptoms.  If  the  tumor  extends  downward  and  involves  the  ol- 
factory bulb  the  sense  of  smell  may  be  diminished  or  lost ;  but  changes  in 
character  and  intelligence,  "punning,"  irritability  or  stupidity,  are  considered 
to  be  relatively  frequent  in  diseases  of  the  frontal  lobe,  and  this  is  entirely  in 
support  of  the  conditions  which  Goltz  first  discovered  as  a  change  in  the  be- 
havior of  dogs  whose  frontal  lobes  were  excised  or  destroyed.  More  recently 
Torje  has  recorded  a  case  in  which  there  was  marked  disturbance  of  saliva- 
tion ;  he  suggests  that  the  drooling  in  idiots  may  be  due  to  defective  frontal 
development.  Bruns  has  mentioned  the  occurrence  of  cerebellar  ataxia  in 
these  cases.  Frontal  tumor  may  also  be  diagnosticated  if  the  general  symp- 
toms were  present  for  a  long  period  of  time,  and  then  symptoms  pointing  to 
the  third  frontal  gyrus  or  the  motor  areas  appear  as  evidence  of  the  gradual 
encroachment  of  the  new-growth  upon  these  areas. 

Tumors  of  the  Third  Frontal  Convolution.— The  third  frontal 
convolution  is  the  seat  of  motor  speech  function,  and  any  destructive  lesion 
in  or  near  this  part  of  the  brain  will  cause  typical  motor  aphasia.  Agraphia 
may  be  associated  with  motor  aphasia.  The  cases  in  which  motor  aphasia, 
or  agraphia,  or  both,  have  signalized  the  invasion  of  a  tumor  are  compara- 
tively rare,  particularly  in  children ;  for  if  the  invasion  of  the  tumor  be  a  grad- 
ual one  the  right  hemisphere  may,  in  right-handed  persons,  gradually  assume 
the  functions  of  speech,  which  up  to  that  time  have  been  inherent  in  the  left 
hemisphere.  In  addition  to  this,  in  young  children  the  differentiation  between 
the  left  and  the  right  hemispheres  is  not  nearly  as  complete  as  in  later  years. 

Tumors  of  the  Motor  Area.— These  can  be  recognized  more  readily 
than  those  in  any  other  part  of  the  cortex.     In  the  earlier  stages  of  the  dis- 

*  If  there  is  excessive  intra-cranial  pressure,  headaches  may  be  intense,  even  in 
tumors  distant  from  the  surface. 


476  THE  NERVOUS  DISEASES   OF  CHILDREN". 

ease  symptoms  of  cortical  irritation  are  present,  and  convulsive  twitchings  of 
the  paretic  or  paralyzed  part  will  indicate  the  probable  site  of  the  tumor. 
Thus,  if  a  patient  with  slight  sensory  and  motor  disturbances  in  the  upper 
extremity  should  experience  occasional  convulsive  twitchings  of  the  arm,  of 
the  forearm,  or  even  of  the  thumb  alone,  the  character  and  extent  of  these 
twitchings  would  point  to  the  part  of  the  brain  first  and  chiefly  involved. 
According  to  the  course  and  development  of  the  convulsive  seizures  the 
direction  in  which  the  growth  extends  can  be  judged.  Vasomotor  symptoms 
and  changes  in  pulse-rate  are  uncommon. 

Tumors  of  the  Parietal  Lobe. — Tumors  of  the  parietal  lobe  may 
cause  no  distinct  localizing  symptoms,  but  the  records  of  Mills  and  Spiller 
prove  conclusively  that  lesions  in  these  parts  are  apt  to  be  followed  by  sen- 
sory changes  (loss  of  muscular  sense,  astereognosis,  etc.).  These  facts  of 
human  pathology  are  in  keeping  with  the  results  of  Munk's  experiments  on 
dogs,  and  those  of  Schafer  and  Horsley  on  monkeys.  These  sensory  symp- 
toms, however,  are  not  so  constant  that  their  absence  would  militate  against 
the  diagnosis  of  tumor  in  this  region.  If  a  tumor  in  the  parietal  lobe  affects 
the  subjacent  white  matter  it  may  result  in  hemianopsia,  for  the  white  tract 
of  Gratiolet  passing  from  the  internal  capsule  to  the  occipital  lobe  lies  under 
this  portion  of  the  cortex.  Ferrier's  claim,  that  the  centre  of  vision  lies  in 
the  angular  gyrus,  is  due  to  the  involvement  by  disease  in  this  region  of  the 
optic  radiations  which  we  have  mentioned.  By  extension  to  the  inferior 
parietal  lobule  the  tumor  may  give  rise  to  that  peculiar  disturbance  of  speech 
known  as  word-blindness.  The  patient  cannot  read  and  write  at  will,  though 
he  may  be  able  to  write  upon  dictation,  or  to  copy  written  signs.  In  children, 
excepting  those  of  a  more  advanced  age,  cases  will  rarely  arise  in  which 
word-blindness  or  word-deafness  are  important  symptoms.  According  to 
Wernicke  the  conjugate  movements  of  the  eyes  are  governed  by  a  centre  in 
the  inferior  parietal  lobule.  If  there  is  distinct  impairment  of  this  one  func- 
tion, disease  in  this  region  may  therefore  be  suspected. 

Tumors  of  the  Occipital  Lobe. — Tumors  in  the  occipital  lobe  are 
recognized  by  the  peculiar  disturbances  of  vision.  As  each  occipital  lobe  is 
connected  with  one-half  of  each  eye,  tumor  in  this  region  is  signalized  by 
homonymous  hemianopsia  without  any  other  special  symptoms  ;  convulsions 
may  occur,  and  not  necessarily  on  account  of  the  direct  extension  of  the 
lesion  into  the  motor  areas,  but  because,  as  has  been  shown  by  Binswanger, 
disease  in  any  part  of  the  cortex  may  cause  epileptic  disturbances.  The 
researches  of  Nothnagel  and  Seguin,  which  have  been  corroborated  by  other 
authors,  points  with  a  great  degree  of  certainty  to  the  cuneus  as  the  actual 
centre  of  vision.  Visual  hallucinations  and  word-blindness  have  been  re- 
corded by  a  number  of  reputable  writers.  According  to  Bruns,  optic  neuritis 
is  relatively  infrequent. 

Tumors  of  the  Temporo-sphenoidal  Lobe. — The  temporo-sphe- 
noidal  lobe  contains  the  centres  for  hearing  and  for  sensory  speech ;  it  is, 
therefore,  natural  to  expect  that  in  the  case  of  tumor  in  these  auditory  re- 
gions hearing  will  be  impaired,  not  abolished,  on  the  side  opposite  the  lesion, 
and  sensory  aphasia  will  be  present.     The  patient  will  be  able  to  speak  spon- 


TUMORS   OF   THE   BRAIN  AND  ITS  MENINGES.  477 

taneously  and  correctly,  but  will  not  be  able  to  understand  what  is  being 
said  to  him,  and  will,  of  course,  not  be  able  to  repeat  spoken  language.  The 
differential  diagnosis  between  tumor  and  abscess  should  be  established  care- 
fully. Deep-seated  tumors  may  cause  hemianaesthesia,  hemiplegia,  and 
hemianopsia. 

Tumors  of  the  Basal  Ganglia. — Tumors  of  the  basal  ganglia  and  the 
adjacent  parts  are  not  rare.  The  symptoms  which  they  produce  are,  as  far 
as  we  know,  entirely  due  to  direct  or  indirect  involvement  of  the  internal  cap- 
sule. As  this  capsule  contains  the  entire  motor  tract  for  the  opposite  side  of 
the  body,  the  sensory  fibres,  the  fibres  of  special  sense,  the  speech-tracts,  we 
can  infer  the  multiplicity  of  symptoms  which  may  result  from  such  a  le- 
sion. The  position  of  these  fibres  in  the  internal  capsule  is  quite  well  es- 
tablished, and  in  the  case  of  small  tumors,  the  order  of  involvement  of  the 
different  functions  may  give  some  clew  as  to  the  direction  in  which  the  tumor 
is  extending.  The  tumor  may  impinge,  too,  upon  the  lateral  ventricles,  and 
thus  cause  considerable  disturbance.  Some  of  the  symptoms  may  be  sec- 
ondary to  the  increase  of  hydrocephalic  fluid.  On  account  of  the  compact 
structure  of  the  inner  part  of  the  brain,  even  small  tumors  will  cause  con- 
siderable distortion  of  the  parts,  and  in  addition  to  all  the  other  symptoms 
presented  as  the  result  of  the  involvement  of  the  internal  capsule,  we  may 
have  symptoms  resulting  indirectly  from  pressure  upon  the  cranial  nerves, 
which  come  off  from  the  brain  axis  below  this  region.  The  differential  diagno- 
sis between  tumors  of  the  ganglia  and  tumors  of  the  cortex  will  be  based  in 
part  upon  the  absence  of  convulsive  seizures  in  the  case  of  ganglionic  tu- 
mors, and  the  more  frequent  presence  of  choreic  or  athetoid  movements 
with  cortical  disease. 

Tumors  of  the  Crus  Cerebri. — A  neoplasm  in  this  region  can  be 
recognized  very  early  by  the  association  of  oculo-motor  symptoms  with  paral- 
ysis of  motion  and  sensation  of  the  opposite  half  of  the  body.  The  eye 
will  exhibit  ptosis,  paralysis  of  all  the  external  muscles  except  the  rectus  ex- 
ternus  and  the  superior  oblique,  and  complete  paralysis  of  the  sphincter 
iridis,  and  the  ciliary  muscle. 

Both  peduncles  are  so  close  to  one  another  that  a  tumor  occurring  in  one 
may  actually  invade  the  opposite  side,  or  at  least  press  against  the  peduncle 
of  the  other  half  of  the  brain.  For  this  reason  it  is  not  uncommon  to  find 
paralysis  of  both  halves  of  the  body,  or  possibly  double  ptosis  and  double 
oculo-motor  symptoms  in  tumors  in  this  region.  In  the  majority  of  cases, 
however,  the  symptoms  are  strictly  unilateral  for  a  long  period,  and  become 
bilateral  later  on. 

Tumors  of  the  Corpora  Quadrigemina. — These  tumors  should 
in  reality  be  considered  with  tumors  of  the  peduncles,  for  these  structures  are 
so  closely  related  to  one  another,  anatomically  and  physiologically,  that  the 
occurrence  of  neoplasm  in  the  one  part  or  the  other  will  produce  a  common 
series  of  symptoms.  It  is  only  the  occurrence  of  oculo-motor  paralysis  with 
opposite  hemipleglia  that  suggests  the  region  of  the  peduncles,  while  a  few 
additional  symptoms  refer  distinctly  to  the  corpora  quadrigemina.  These 
additional  symptoms  are  due  to  the  relation  which  the  corpora  quadrigemina 


478 


THE  NERVOUS  DISEASES   OF  CHILDREN. 


bears  to  visual  functions,  and  to  the  connection  between  the  former  and  the 
cerebellum.  Loss  of  pupillary  reflexes,  nystagmus,  vertigo,  and  a  condition 
resembling  cerebellar  ataxia,  point  to  the  region  of  the  corpora  quadrigemina 
as  the  special  seat  of  the  lesion.  I  had  occasion  a  few  years  ago  to  publish 
an  interesting  case  of  this  description,  with  the  result  of  the  post-mortem 
examination  (Fig.  120).  Tumors  of  the  pineal  gland  will  cause  an  entirely 
similar  series  of  symptoms.  Marked  vasomotor  disturbances  have  been 
recorded  by  Collins  and  others  with  neoplasm  in  this  region. 

Tumors  of  the  Pons. — Tumors  of  the  pons  and  medulla,  like  other  le- 
sions in  these  parts,  give  rise  to  a  great  multiplicity  of  symptoms.  The  pyram- 
idal tracts  are  closely  approx- 
imated to  one  another  near  the  ^«m*r~V'  ^ 
median  line,  so  that,  though  the 
symptoms  may  be  unilateral, 
they  are  often  bilateral.  In  ad- 
dition, therefore,  to  the  hemiple- 
gia, or  double  hemiplegia,  we 
may  have  other  symptoms  point- 
ing to  an  involvement  of  the 
various  cranial  nerves.  A  neo- 
plasm in  the  upper  half  of  the 
pons  may  encroach  more  upon 
the  peduncles  than  upon  the 
structures  of  the  pons  itself,  re- 
sulting in  a  hemiplegia  of  one 
side  of  the  body  with  an  in- 
volvement of  the  third  and  fifth 
nerves  of  the  opposite  side.  If 
the  tumor  is  in  the  lower  half 
of  the  pons,  the  fifth,  sixth,  sev- 
enth, and  eighth  nerves  will  be 
more  or  less  involved,  and  the 
symptoms  resulting  from  this 
affection  will  be  paralysis  of  the 
rectus  externus,  paralysis  of  all 
the  branches  of  the  seventh  nerve 
in  one  half  of  the  face,  and  a  loss 

of  hearing  in  one  ear.  All  these  cranial  nerve  symptoms  will  be  on  the  side  of 
the  lesion  opposite  the  hemiplegia.  If  the  sixth  nerve  nucleus  is  affected  there 
will  be,  in  addition,  distinct  paralysis  of  one  rectus  externus  muscle,  and  pa- 
ralysis of  the  conjugated  movements  of  the  eyes  toward  the  side  of  the  lesion, 
for  this  nucleus  is  connected  with  the  third  nerve  nucleus  of  the  opposite  side 
and  governs  the  outward  movement  of  each  eye.  In  spite  of  this  conjugate 
paralysis  each  internal  rectus  if  examined  separately  may  exhibit  normal 
movements.  If  the  lesion  is  near  the  surface  of  the  pons,  and  away  from  the 
nucleus,  it  will  involve  the  root  of  the  sixth  nerve,  and  will  cause  paralysis  of 
the  rectus  externus  muscle  of  one  side,  but  will  not  affect  the  conjugate 


Fig.  120. — Section  passing  through  the  Pos- 
terior Quadrigeminal  Bodies.  T,  tumor  (sol- 
itary tubercle)  ;  P.  Q,  posterior  quadrigemi- 
nal body. 


TUMORS   OF  THE  BRAIN  AND  ITS  MENINGES.  479 

movements  of  the  opposite  side.  If  the  patient  is  directed  to  look  toward 
the  side  of  the  paralyzed  rectus  or  toward  the  side  of  the  tumor  the  opposite 
eye  will  move  promptly,  the  affected  eye  remaining  fixed.  This  differentia- 
tion between  the  isolated  paralysis  of  the  rectus  externus  and  paralysis  of  the 
conjugated  movements  is  the  most  valuable,  and  perhaps  the  only  differen- 
tial point  of  diagnosis  that  helps  at  times  to  distinguish  between  a  tumor 
near  the  surface  and  one  within  the  substance  of  the  pons. 

The  difficulties  of  diagnosis  of  pons  lesions  are  increased  by  the  exceeding 
variability  of  the  symptoms,  and  by  the  fact  that  some  of  the  nerves  in  the 
pons  escape  disease,  whereas  others  may  be  intensely  affected. 

In  the  case  of  a  tumor  in  the  medulla  oblongata  the  symptoms  are  very 
similar  to  those  met  with  in  bulbar  palsy.  The  symptoms  will  indicate  dis- 
ease of  the  glosso-pharyngeal,  of  the  vagus,  of  the  spinal  accessory,  and  of 
the  hypoglossal  nerves,  together  with  a  unilateral  or  bilateral  paralysis  of  the 
arms  or  legs  ;  the  facial  nerve  will  not  be  involved.  Difficulties  in  degluti- . 
tion,  in  respiratory  and  cardiac  movements ;  paralysis  or  spasm  of  the  sterno- 
cleido  mastoid,  and  of  the  trapezius ;  paralysis  and  atrophy  of  the  tongue, 
together  with  vomiting,  with  glycosuria  or  polyuria — all  these  signs  will 
suggest  a  lesion  in  the  medulla.  Tumors  in  this  region,  and  particularly 
gummata,  are  not  rare.  Difficulties  in  diagnosis  often  arise  on  account  of 
the  bilateral  character  of  all  the  symptoms,  but  it  should  be  remembered  that 
the  two  halves  of  the  brain  at  this  level  are  scarcely  separated  from  one  an- 
other, and  that  it  is  natural  for  all  but  vascular  lesions  (and  even  these  may 
do  so)  to  produce  bilateral  symptoms.  In  the  pons  and  medulla  the  sensory 
tracts  also  lie  very  closely  to  the  motor,  and  for  this  reason  the  symptoms 
may  be  still  further  complicated  by  the  occurrence  of  partial,  or  complete,  or 
double  hemianesthesia.  As  the  cerebellum  is  also  in  close  juxtaposition  to 
the  medulla  and  the  pons,  symptoms  of  cerebellar  involvement  may  be  pres- 
ent in  the  cases  of  neoplasms,  both  in  the  pons  and  in  the  medulla.  Optic 
neuritis  is  developed  at  a  very  early  day,  and  occipital  headaches  are  par- 
ticularly severe. 

Tumors  of  the  Cerebellum. — Tumors  of  the  cerebellum  are  the 
most  frequent.  The  symptoms  are  occipital  or  frontal  headache,  projectile 
vomiting,  rigidity  of  the  neck,  and  (at  a  relatively  early  period)  a  peculiar 
staggering  or  reeling  gait  (cerebellar  titubation).  Double  optic  neuritis  has 
been  found  in  eighty-nine  per  cent,  of  these  cases.  Inco-ordination  is 
frequent.  Asthenia  or  muscular  weakness  is  a  real  cerebellar  symptom 
(Mills).  Asynergia  or  hemiasynergia  (Babinski) — asynchronous  movement  of 
the  thigh  and  leg  in  extending  the  limb — is  of  questionable  value.  Vertigo  is 
also  more  frequent  and  more  severe  in  cerebellar  tumors  than  in  many  others. 
In  my  own  rather  extensive  experience  I  have  found  that  the  patient  is  apt  to 
fall  toward 'the  side  on  which  the  tumor  is  located.  Mills  believes  that  destruc- 
tive and  irritative  lesions  of  the  vermis  are  responsible  for  a  tendency  to  fall 
forward  or  backward.  A  cerebellar  tumor  may  be  of  very  small  size  and  yet 
sufficiently  large  to  compress  the  sixth,  seventh,  and  eighth  nerves,  and  in  its 
further  growth  may  produce  involvement  of  the  bulbar  nerves  as  well,  just  as 
tumor  of  the  bulb  may  also  produce  cerebellar  symptoms.     The  affection  of 


48o 


THE   NERVOUS  DISEASES   OF   CHILDREN". 


the  sixth  nerve,  causing  paralysis  of  the  rectus  externus,  is  extremely  common 
in  cerebellar  tumors,  and  in  some  cases  the  diagnosis  of  cerebellar  tumor  may 
be  corroborated  by  the  early  involvement  of  the  seventh  and  eighth  nerves 
(Fig.  1 21). 

Of  late  years,  Monakow,  Sternberg,  Oppenheim,  Fraenkel,  and  Hunt  have 
called  attention  to  slowly  growing  tumors  (neuromata  and  neurofibromata) 
in  the  cerebello-pontile  angle,  involving  one  or  more  cranial  nerves,  especially 
both  auditory  nerves.  Cerebellar  symptoms  are  preceded,  often  for  many 
years,  by  those  pointing  to  involvement  of  the  auditory,  the  trigeminal,  the 


Fig.   121. — Tumor  (Sarcoma)  of  the  Cerebello-Pontile  Angle, 
of  facial  and  auditory  nerve  symptoms. 


Early  appearance 


vagus,  of  one  or  both  sides.  I  have  under  observation  at  the  present  time  a 
young  girl  who,  for  years  past,  has  suffered  from  central  deafness,  ataxia  of 
the  right  upper  extremity,  dizziness,  and  who  now  is  also  completely  blind 
from  double  optic  neuritis. 

The  general  manifestations  of  cerebellar  tumors  do  not  vary  much  from 
tumors  in  other  parts  of  the  brain ;  but  if  the  growth  encroaches  upon  the 
pyramidal  tracts  in  the  pons  and  medulla  unilateral  or  bilateral  paralysis  will 
follow.  The  reflexes  are  sometimes  exaggerated,  as  in  all  other  cerebral 
diseases,  but  in  not  a  few  cases  the  knee-jerks  are  either  diminished  or 
absent. 


Differential  Diagnosis. — The  diagnosis  between  tu- 
mor and  abscess  will  depend  upon  the  frequent  presence  cf 
fever,  and  upon  the  very  slow  invasion  and  slow  develop- 
ment of  all  the  symptoms  in  the  latter  condition,  also  upon 
the  occasional  absence  of  optic  neuritis  in  spite  of  the 
presence  of  symptoms  pointing  to  increased  intra-cranial 


TUMORS   OF  THE  BRAIN  AND  ITS  MENINGES.         48 1 

pressure;  but  the  differential  diagnosis  will  depend  first  and 
foremost  upon  the  proof  of  the  presence  of  such  conditions 
which  give  rise  to  abscess,  or  the  absence  of  such  conditions 
in  the  case  of  tumor.  For  other  symptoms  characteristic  of 
abscess  rather  than  of  tumor,  the  student  is  referred  to  the 
next  chapter. 

The  ordinary  forms  of  tubercular  meningitis  are  so  dis- 
tinct that  they  cannot  be  readily  confounded  with  tumor  of 
the  brain,  but  the  difficulty  lies  in  the  fact  that  some  cases 
of  tubercular  affection  of  the  brain  take  an  exceedingly  slow 
course,  and  that  some  forms  of  tumor  prove  rapidly  fatal. 
Furthermore,  tumor  in  the  form  of  solitary  tubercle  and 
meningitis  are  occasionally  associated  with  one  another,  as 
was  proved  in  a  case  of  mine  of  solitary  tubercle  of  the 
corpora  quadrigemina.  In  meningitis,  whether  of  a  tuber- 
cular character  or  not,  the  rapid  involvement  of  a  number 
of  cranial  nerves,  without  an  increase  in  all  the  other  symp- 
toms, will  argue  in  favor  of  this  disease  rather  than  of 
tumor.  This  is  true  also  of  the  specific  meningitis  as  well 
as  of  the  tubercular  form.* 

Histological  examination  of  the  cerebro-spinal  fluid,  obtained  by  lumbar 
puncture,  may  assist  in  the  diagnosis.  Beware  of  lumbar  puncture  in  cere- 
bellar disease !  The  X-rays  may  occasionally  be  of  service  in  locating  a  new 
growth  (Mills,  myself  and  others). 

Pathology.— The  most  important  intra-cranial  growth 
is  the  solitary  tubercle.  It  occurs  both  in  children  who  ex- 
hibit distinct  tubercular  tendencies,  and  also  in  those  who 
neither  give  a  family  history  of  tuberculosis  nor  exhibit 
any  cachectic  symptoms. 

In  size  the  solitary  tubercle  may  vary  from  that  of  a  pea  or  cherry  to  one 
as  large  as  that  depicted  in  Fig.  122.  In  this  brain  it  will  be  seen  that  a 
solitary  tubercle  had  occupied  the  greater  part  of  one  hemisphere,  and  had 
considerably  distorted  the  entire  brain  axis.  Very  often  the  tumor  starts  in 
the  vicinity  of  the  meninges,  or  at  least  near  the  cortical  blood-vessels.  The 
blood-vessels  unquestionably  play  an  important  part,  for  in  cases  of  tuber- 

*  Oppenheim  has  reported  several  interesting  cases  of  children  presenting  all  the 
symptoms  of  a  tumor  of  the  Rolandic  area,  and  ending  in  recovery.  He  thinks  these 
may  have  been  cases  of  meningo-encephalitis  tuberculosa.  Nonne  has  also  described 
cases  in  adults  and  in  youthful  individuals  supposed  to  be  suffering  from  tumors  of  the 
brain ;  all  symptoms  had  receded.  Hydrocephalus,  serous  meningitis,  and  specific 
lesions  may  cause  difficulties  in  diagnosis. 


482 


THE  NERVOUS  DISEASES   OF  CHILDREN. 


cular  meningitis  of  the  base  smaller  tubercles  of  a  pin-head  size  may  be  seen 
scattered  along  the  vessels  on  the  outer  surface  of  the  brain.  A  solitary- 
tubercle  is  composed  of  a  thick  stroma  with  giant  cells  and  a  considerable 
amount  of  hard  fibrous  tissue.  There  is  a  more  or  less  concentric  arrange- 
ment of  the  parts,  and  the  central  portion  of  the  tumor,  in  many  cases,  un- 
dergoes caseous  degeneration  at  an  early  day.  As  in  the  cord,  so  in  the  brain, 
the  tissue  surrounding  a  tubercular  growth  may  break  down  and  become  an 
almost  diffluent  mass.  A  tubercular  encephalitis,  or  meningo-encephalitis, 
may,  therefore,  be  superadded  upon  a  solitary  tubercle.  In  just  as  many 
cases,  however,  the  solitary  tubercle  behaves  as  every  other  cerebral  tumor 
does.     It  is,  as  a  rule,  sharply  differentiated  from  the  surrounding  tissue,  and 


Fig.  122. — Vertical  Section  through  Cyst,  C,  and  Both  Tumors,  showing  Distortion  of 
Brain  Axis  and  Displacement  of  Left  Ventricle.     P,  pons  ;   l\  the  left  ventricle. 


the  exact  character  of  it  can  be  made  out  only  on  histological  examination. 
The  very  size  of  the  tumor,  as  in  the  brain  depicted  in  Fig.  122,  is  suspi- 
cious of  solitary  tubercle,  for  the  smallest  as  well  as  the  largest  tumors  occur- 
ring in  the  brain  are  of  this  character.  In  the  histological  examination  of  the 
tumor  search  should  be  made  for  the  tubercle  bacilli,  and  their  presence  will 
at  once  prove  or  disprove  the  supposed  character  of  the  tumor. 

Glioma  and  sarcoma,  or  a  combination  of  both,  are  the 
next  most  frequent  forms  of  neoplasm  occurring  in  the  brains 
of  children.  The  glioma  possesses  the  characteristics  of  neu- 
roglia tissue.  Its  growth  is  extremely  slow,  and  for  this 
reason  it  may  be  suspected  in  cases  in  which  all  the  symp- 
toms point  to  a  period'  of  growth  lasting  for  one  or  two 


TUMORS    OF   THE   BRAIN  AND  ITS  MENINGES.  483 

years,  or  even  a  longer  period  of  time.  In  connection  with 
glioma,  as  in  other  forms  of  tumor,  there  is,  as  a  rule,  a  con- 
siderable increase  in  the  blood-supply  to  surrounding  parts. 
An  increase  of  cerebro-spinal  fluid  is  a  constant  accompani- 
ment, and  it  must  be  due  to  variations  in  the  amount  of 
these  fluids  that  improvement  sometimes  follows  upon  treat- 
ment by  iodides  and  mercurials. 

A  patient  of  mine  had  been  suffering  for  a  long  time  from  symptoms  point- 
ing to  a  tumor  in  the  parietal  region ;  he  had  also  had  an  apoplectic  attack  some 
years  previously.  Taking  these  facts  into  consideration  I  concluded  that  the 
tumor  was  probably  of  a  specific  character,  and  placed  him  upon  a  very  rigid 
iodide  and  mercurial  treatment.  The  improvement  was  most  marked,  the 
headaches  ceased,  the  convulsive  seizures  disappeared  altogether,  and 
the  power  in  the  right  hand  returned.  I  was  convinced  of  the  specific 
character  of  the  tumor.  The  patient  died,  however,  in  a  relapse,  accompanied 
by  an  apoplectic  seizure,  and  to  my  great  surprise,  on  post-mortem  examina- 
tion, the  tumor,  which  was  examined  by  Dr.  Van  Gieson,  proved  to  be  a 
glioma  and  not  a  gumma.  It  is  of  the  utmost  importance,  therefore,  not  to 
attach  too  great  significance  to  any  improvement  occurring  in  these  diseases 
after  specific  treatment. 

Gliomata  occur  more  frequently  in  the  white  than  in  the  gray  matter  of  the 
brain,  but  may  invade  the  latter  though  starting  from  the  white  substance. 
The  pons  shares  with  the  hemispheres  the  distinction  of  being  the  most  fre- 
quent seat  of  glioma,  the  dense  character  of  the  tissue  in  the  pons  evidently 
yielding  favorable  conditions  for  the  development  of  this  special  form  of 
neoplasm. 

Sarcoma,  pure  and  simple,  or  in  connection  with  glioma,  is  somewThat  less 
frequent  than  glioma  alone.  Its  growth  is  more  rapid  than  that  of  simple 
glioma,  and  it  is  not  as  distinctly  separated  from  the  healthy  tissue  as  glioma 
generally  is  ;  the  tumor  and  infiltration  as  a  rule  extend  for  some  distance  into 
the  neighboring  tissue.  By  this  invasion  and  by  actual  compression  much 
of  the  tissue  in  the  vicinity  of  a  sarcoma  is  readily  broken  down.  Round 
cells  and  spindle-shaped  cells  occur  together  with  the  fibrous  mass,  and 
according  to  the  predominating  character  of  the  cells  the  sarcomata  are  di- 
vided into  round-celled  and  spindle-celled  varieties.  Myxo-sarcoma  is  not 
unknown  in  childhood,  and  a  few  cases  have  been  reported  of  that  much 
rarer  form  known  as  melano-sarcoma.  The  sarcomata  are  invariably  secon- 
dary to  similar  diseases  in  other  organs.  Sarcoma  may  therefore  be  sus- 
pected with  malignant  disease  in  other  parts  of  the  body,  and  particularly  if 
the  tumor  be  one  of  rapid  growth,  and  if  the  symptoms  point  to  an  intensely 
destructive  process. 

Cysts  are  extremely  frequent  in  children,  and  are  often  found  in  brains 
not  suspected  of  any  gross  disease.  Such  cysts  are  generally  the  remnants 
of  an  acute  process  early  in  life,  an  early  hemorhage,  or  a  softening  due  to 


484  THE  NERVOUS  DISEASES   OE   CHILDREN. 

embolism  or  thrombosis.  These  cysts  fill  up  with  fluid,  and  cause  in  the 
majority  of  cases  no  symptoms  such  as  we  assign  to  tumor ;  but  if  from  any 
cause  such  an  old  cyst  is  lighted  up  and  its  contents  suddenly  increase,  the 
symptoms  may  resemble  those  of  cerebral  neoplasm.  It  is  well  to  be  pre- 
pared for  the  occurrence  of  such  cysts,  and  to  know  that  they  are  a  favorite 
starting-point  for  other  tumors.  It  was  on  the  strength  of  such  familiarity 
with  the  cerebral  conditions  of  childhood  that  I  was  able  to  make  the  diag- 
nosis of  a  tumor  growing  from  the  cyst-walls  in  the  case  referred  to  on  page 
569. 

Cystic  tumors,  in  the  true  sense  of  the  word,  are  those  which  are  due  to 
an  invasion  of  echinococcus.  In  this  country  it  is  extremely  rare  to  find  a 
tumor  of  this  description,  but  in  Europe  and  in  Eastern  countries  they 
are  far  more  frequent.  The  cysticercus  cysts  occur  in  children  as  well  as  in 
adults,  are  multiple,  and  may  give  rise  to  a  confusion  of  symptoms. 

The  only  other  form  of  neoplasm  which  deserves  special 
consideration  is  that  due  to  syphilitic  disease  Gummata 
are  unquestionably  rare  in  children,  but  they  may  of  course 
occur  in  cases  of  hereditary  syphilis.  We  should  look  for 
them  in  connection  with  specific  disease  of  the  brain  and 
its  coverings.  Repeated  attacks  and  relapses  would  sug- 
gest the  syphilitic  character  of  the  disease. 

Aneurism  is  also  so  rare  in  childhood  that  it  would  hardly  need  to  be 
considered.  But  Osier  reported  some  years  ago  an  interesting  case  of  aneu- 
rism in  a  child  six  years  of  age.  The  symptoms  are  very  much  like  those 
caused  by  ordinary  neoplasm,  but  the  course  of  the  disease  is  much 
more  chronic  than  in  the  case  of  other  new-growths.  The  diagnosis  of 
aneurism  could  be  reached  only  by  exclusion,  by  the  slow  course  of  the  dis- 
ease, and  possibly  by  irregularities  of  the  pulsations  of  the  two  carotid  arte- 
ries. The  anterior  and  posterior  cerebral  arteries  are  the  vessels  favored  by 
aneurisms  of  any  considerable  size. 

In  concluding  these  remarks  upon  the  pathology  of 
tumors,  it  is  well  to  remind  the  student  once  more  that  the 
symptoms  produced  by  any  tumor  are  in  part  due  to  the 
tumor  itself,  to  the  character  of  the  invasion,  and  to  the 
region  of  the  brain  affected,  and  in  part  to  the  pressure 
upon  the  neighboring  tissues,  and  upon  distant  parts  of  the 
brain  structure.  The  increase  of  intra-cranial  fluid  plays  a 
most  important  part  in  the  causation  of  some  of  the  symp- 
toms, notably  of  headaches  and  of  optic  neuritis. 

Treatment. — In  spite  of  many  advances  in  recent  years, 
the  medical  or  surgical  treatment  of  brain  tumors  has  been 


TUMORS   OF  THE  BRAIN  AND  ITS  MENINGES.  485 

disappointing.  According  to  Starr's  statistics  only  7.5  per 
cent,  of  the  cases  are  operable.  Ferrier  and  Oppenheim 
give  a  similar  percentage.  Bramwell  thought  many  years 
ago  five  out  of  twenty-two  cerebral  tumors  could  have  been 
removed.  In  children  the  prognosis  is  still  gloomier.  For 
this  there  is  a  double  reason;  first,  tubercle  is  very  much 
more  frequent  in  children  than  in  adults;  second,  if  the  con- 
ditions for  the  removal  of  the  tumor  are  favorable,  the  child 
stands  a  poorer  chance  of  surviving  the  operation  than  the 
adult  does.  Before  proceeding  to  the  surgical  treatment  of 
the  case  of  brain  tumor,  which  is,  after  all,  the  only  thor- 
oughly rational  course,  the  attempt  should  be  made  to  in- 
fluence the  growth,  and  to  promote  the  absorption  of  the 
new-growths  by  various  drugs.  The  most  powerful  drugs 
are  the  iodides  and  the  mercurials.  Gummata  are  very 
rare  in  children,  and  this  treatment  would  therefore  be  of 
little  use  if  it  were  given  for  its  anti-syphilitic  effect.  But 
in  every  case  of  tumor  there  is  considerable  exudation,  con- 
siderable increase  of  cerebro-spinal  fluid,  and  both  the 
iodides  and  the  mercurials  seem  to  have  some  effect  upon 
the  absorption  of  these  products  of  inflammatory  reaction 
in  connection  with  tumor.  By  promoting  such  absorption 
of  adventitious  products  the  intense  localized  headaches 
and  persistent  vomiting,  and  other  general  cerebral  symp- 
toms frequently  present,  are  modified.  Another  reason  for 
the  employment  of  specific  remedies  is  the  belief  that  if  they 
will  not  avail  any  in  the  actual  cure  of  the  trouble,  they  will 
at  least  help  to  relieve  some  of  the  most  distressing  symp- 
toms that  occur  in  connection  with  cerebral  neoplasm.* 

If  any  decided  effect  is  to  be  hoped  for,  the  iodides,  as  well  as  the  mercu- 
rials, should  be  given  in  effective  doses  ;  the  iodides  in  doses  varying  from 
five  to  twenty-five  grains  three  times  a  day ;  the  mercury,  in  the  form  of 
inunctions,  from  fifteen  to  thirty  and  forty-five  grains  twice  a  day,  according 
to  the  age  of  the  child.     All  other  medicinal  treatment  will  be  of  no  avail. 

The  headaches  will  be  relieved  best  by  a  combination  of  phenacetine  with 
codeine  (one-fourth  to  one-third  of  a  grain),  or  with  small  doses  of  morphia 
(one-sixteenth  to  one-eighth  of  a  grain),  according  to  the  age  of  the  child. 
Morphia  alone  I  am  not  in  favor  of  giving ;  and  if  the  pains  are  so  extreme 

*  Lumbar  puncture  might  be  tried  in  inoperable  cases  for  the  relief  of  pressure. 
Trephining  for  the  relief  of  pressure  is  justifiable  and  beneficial. 


486  THE  NERVOUS  DISEASES   OE  CHILDREN. 

that  the  child  becomes  restless  and  sleepless  in  consequence  of  them,  I  should 
prefer  moderate  doses  of  chloral  hydrate,  from  five  to  ten  grains,  according 
to  the  age  of  the  child,  given  either  per  mouth  or  per  rectum.  If  the  tumor 
causes  frequent  convulsions  the  bromides  might  be  added  to  the  chloral,  but 
it  is  best  to  avoid  them  in  all  cases  in  which  epileptic  seizures  do  not  occur, 
for  the  bromides  have  no  effect  upon  the  intense  headaches,  unless  given  in 
very  large  doses,  and  have  a  disagreeable  effect,  interfering  with  the  diges- 
tion and  nutrition  of  the  child.  The  ordinary  hypnotics,  such  as  sulfonal, 
trional,  veronal,  and  the  like,  can  be  employed  in  moderate  doses  in  those 
cases  in  which  there  is  a  general  restlessness  without  pain. 

In  the  first  edition  of  this  book  the  author  took  a  dis- 
tinctly pessimistic  view  of  the  surgical  treatment  of  brain 
tumors.  Within  the  last  few  years  the  brilliant  work  of 
Horsley,  of  Kocher,  of  Keen,  and  the  recent  enthusiastic 
and  ingenious  writings  of  Frazier  and  of  Cushing  have 
given  promise  of  far  greater  successes  in  this  special  field. 
Even  the  cerebellum  has  been  made  accessible.  Sanger  and 
Cushing  are  right  in  urging  the  great  value  of  a  palliative 
craniectomy  as  a  means  of  "decompression"  for  the  relief 
of  headaches,  and  for  the  preservation  of  the  eyesight. 
Within  the  last  year  this  palliative  operation  has  been  per- 
formed for  me  by  Gerster  or  Lilienthal  in  four  cases  of  cere- 
bellar tumor  occurring  in  children.  The  difficulties  of  sur- 
gical interference  in  children  are,  in  part,  similar  to  those 
met  with  in  the  adult,  and  in  part  peculiar  to  children  only. 
Among  the  first  we  may  mention  the  uncertainty  which  still 
prevails  in  every  case  regarding  the  exact  size  of  a  tumor, 
particularly  if  the  tumor  begins  in  one  of  the  active  areas  of 
the  brain  and  spreads  to  a  so-called  silent  area.  On  this  ac- 
count alone,  however,  no  one  would  be  justified  in  refrain- 
ing from  an  operation;  this  uncertainty  constitutes  one  of 
the  attending  risks. 

Among  the  conditions  peculiar  to  children  I  would  mention  first  of  all  the 
fact  that  children,  according  to  my  experience,  which  has  been  quite  exten- 
sive, tolerate  operations  upon  the  brain  very  poorly  indeed.  The  cerebral 
shock,  and,  above  all,  the  considerable  cerebral  hemorrhage,  lessen  the  chances 
of  recovery  very  much.  *  If  we  consider,  moreover,  that  a  very  large  number 
of  the  cerebral  tumors  in  children  are  of  a  tubercular  nature,  that  multiple 

*  The  student  is  referred  to  the  interesting  articles  of  Frazier  (N.  Y.  Med.  Journal, 
February  n  and  18,  1905)  and  of  Harvey  Cushing  (The  Johns  Hopkins  Hospital 
Bulletin  for  March,  1905). 


TUMORS   OF   THE   BRAIN  AND   ITS  MENINGES.  487 

tumors  are  not  infrequent,  that  the  tumors  occur  with  great  frequency  in 
the  cerebellum  and  on  the  ventral  surface  of  the  brain,  we  are  forced  to  the 
conclusion  that  there  are  but  few  cases  of  intra-cranial  tumor  in  children 
which  seem  favorable  for  operation,  and  that  of  these  few  it  must  be  a  rare 
fortune  indeed  to  have  a  single  one  recover  completely.  It  is  the  duty  of 
every  careful  physician  to  weigh  the  evidence  for  and  against  an  operation  in 
every  case,  and  if  in  a  child  of  good  physical  strength  a  non-tubercular  tumor 
is  suspected  in  a  region  accessible  to  the  surgeon's  knife,  the  attempt  at  re- 
moval of  such  a  tumor  is  justifiable  if  all  other  remedial  measures  have  been 
given  a  previous  trial.  Whether  a  tubercular  tumor  should  be  removed  or 
not  is  a  question  that  cannot  be  decided  off-hand.  The  answer  may  possibly 
be  given  some  day  by  the  results  of  operation  in  a  case  in  which  the  character 
of  the  tumor  was  not  suspected  until  it  had  been  successfully  or  unsuccessfully 
removed.  * 

*  v.  Beck  has  reported  a  case  of  tubercle  of  the  brain  successfully  removed  by 
Czerny.  The  author  reports  improvement  after  the  operation.  Some  years  ago  Dana 
exhibited  a  boy  of  about  12  years  old  who  had  done  well  for  more  than  a  year  after  the 
partial  removal  of  a  sarcoma. 


CHAPTER   XXVII. 

ABSCESS   OF   THE   BRAIN. 

An  abscess  may  be  situated  either  on  the  surface  of  the 
hemispheres,  or  within  the  substance  of  the  brain.  The 
latter  location  is  by  far  the  more  frequent.  Multiple  absces- 
ses sometimes  occur.  Bergmann  found  one  hundred  or  more 
abscesses  in  a  single  case  following-  gangrene  of  the  leg.  In 
one  case  the  author  observed  at  least  six  abscesses,  which 
were  successfully  incised  ;  but  a  single  abscess  is  more  com- 
mon. If  the  pus  is  on  the  surface  of  the  brain  the  mem- 
branes, which  are  generally  thickened  as  the  result  of  the  in- 
flammatory process,  may  constitute  one  wall  of  the  abscess. 

Abscess  of  the  brain  may  occur  at  any  period  of  life.  Of  the  cases  which 
Gowers  has  tabulated  fully  one-third  occurred  before  the  age  of  nineteen. 
The  male  sex  is  more  disposed  to  this  disease  than  females,  in  a  ratio  varying 
from  two  to  one  to  five  to  one,  according  to  the  initial  cause.  The  most 
frequent  cause  of  abscess  of  the  brain  is  preceding  ear  disease.  Next  in  fre- 
quency is  external  injury  to  the  skull.  Gull  and  Sutton,  in  their  article  on 
abscess  of  the  brain,  in  Reynolds's  "  System  of  Medicine,"  state  that  102 
cases  of  abscess  were  due  to  ear  disease,  and  57  to  injury.  Koerner,  in  his 
exhaustive  monograph  on  the  otitic  diseases  of  the  brain,  quotes  Pitt's  statis- 
tics, who  records  56  cases  of  brain  abscess  among  9,000  autopsies.  Of  these 
56  abscesses  18  (very  nearly  one-third)  were  due  to  disease  of  the  ear  and  of 
the  petrous  bone,  while  8  (one-seventh  of  the  entire  number)  were  due  to 
disease  of  other  cranial  bones.  The  relative  number  of  other  cerebral  con- 
ditions complicating  ear  disease,  as  given  by  Koerner,  is  as  follows :  Phle- 
bitis and  thrombosis  of  the  sinuses  occurred  in  44  cases  among  9,000  au- 
topsies, and  of  these  44  fully  one-half  were  due  to  disease  of  the  bony  parts 
of  the  ear.  A  simple  otitic  meningitis  as  the  result  of  purulent  ear  disease 
is  relatively  rare.  Taking  all  cases  of  cerebral  disease  complicating  otitis 
into  consideration  Koerner  states  that  of  246  cases  which  he  has  been  able 
to  collect,  44  occurred  before  the  age  of  ten,  and  73  between  the  ages  of 
eleven  and  twenty  years.  Thus  it  will  be  seen  that  fully  one-third  of  all 
abscesses  occurred  during  the  period  of  childhood  and  early  youth.     Koerner 


ABSCESS    OF   THE   BRAIN.  489 

states,  basing  his  assertions  upon  Prussian  statistics,  that  5.15  per  cent,  of 
all  deaths  between  the  ages  of  ten  and  twenty  were  due  to  cerebral  disease 
complicating  otitis.  These  statistics  will  suffice  to  indicate  the  great  inter- 
est that  must  attach  to  suppurative  disease  in  early  life,  and  particularly  to 
abscess  of  the  brain  following  upon  disease  of  the  ear.  Other  probable 
causes  of  abscess  must  not  be  neglected.  Disease  of  the  nose,  empyema  of 
the  chest,  and  accidental  injury  to  the  skull,  whether  or  not  it  has  given  rise 
to  a  tangible  bone  lesion,  may  be  the  starting-point  of  brain  abscess. 

Gowers  states  that  fifteen  per  cent,  of  all  the  cases  of  abscess  of  the  brain 
were  due  to  what  he  calls  distant  influences.  Among  these  any  condition 
giving  rise  to  a  general  infection  may  be  included,  and  it  is  more  than  prob- 
able that  even  among  this  fifteen  per  cent,  of  cases  some  may  have  been 
traumatic,  although  the  exact  character  of  the  traumatism  could  not  be  de- 
termined. 

In  abscess  of  the  brain  following  upon  chronic  otitic 
disease  the  ear  trouble  may  have  existed  for  several  years.  I 
have  known  such  ear  disease  to  continue  for  ten  years  before 
the  symptoms  of  abscess  were  developed.  As  long  as  there 
is  a  free  discharge  of  pus  outward,  the  danger  of  cerebral 
disease  is  comparatively  slight ;  but  if  the  discharge  is 
checked,  the  pus  may  burrow  its  way  through  the  thin 
plate  of  bone  separating  the  middle  ear  and  the  mastoid 
cells  from  the  cranial  cavity.  The  small  veins  which  pass 
through  the  ear  structures  into  the  cerebral  sinuses  (supe- 
rior petrosal  and  the  lateral  sinus)  provide  easy  means  of 
access  for  the  pus  from  the  inner  ear  into  the  cranial  cavity. 
From  the  mastoid  cells  the  pus  passes  most  easily  into  the 
lateral  sinus.  In  connection  with  ear  disease  the  abscess 
generally  forms  either  in  the  temporo-sphenoidal  lobes  or 
in  the  hemispheres  of  the  cerebellum.  Usually  it  affects 
these  regions,  on  the  same  side  of  the  brain.  In  rare  in- 
stances the  chief  formation  of  pus  is  on  the  side  opposite  to 
the  diseased  ear. 

The  abscess  formed  in  connection  with  ear  disease  is 
more  often  underneath  the  gray  matter  of  the  cortex  than 
upon  the  outer  surface  of  the  hemispheres,  and  not  infre- 
quently normal  tissue  separates  the  abscess  from  the  carious 
bone  and  the  meninges.  Such  being  the  case,  it  is  evident 
that  the  abscess  must  have  been  formed  within  the  brain 
and  gradually  extended  outward.  In  a  few  instances  a  di- 
rect path  of  connection  between  the  suppuration  in  the  ear 


49°  THE  NERVOUS  DISEASES    OF   CHILDREN. 

and  the  abscess  cavity  has  been  demonstrated,  but  in  the 
vast  majority  of  cases  no  such  connection  has  existed.  The 
problem,  therefore,  is  to  decide  in  what  manner  the  pus- 
corpuscles  could  have  been  carried  from  the  ear  to  the  in- 
terior of  the  brain.  No  entirely  satisfactory  solution  has 
as  yet  been  given,  but  the  frequent  interference  with  the 
circulation  in  the  sinuses  undoubtedly  plays  an  important 
part.  If  we  take  into  account  the  relative  position  of  the 
temporo-sphenoidal  lobes  and  the  structures  of  the  ear  it 
will  be  seen  that  the  superior  petrosal  sinus  receives  blood 
from  both  these  parts,  and,  on  the  other  hand,  the  lateral 
sinus  receives  blood  from  the  cerebellum  and  the  mastoid 
cells.  It  is  due  to  these  venous  connections  that  abscess  of 
the  cerebellum  is  met  with  more  frequently  in  connection 
with  mastoid  disease,  and  abscess  in  the  temporo-sphenoidal 
lobes  more  frequently  with -disease  of  the  middle  ear.  Such 
abscesses  have  been  reported  by  Lucae,  Koerner,  Bruns,  Mac- 
ewen,  Gruening,  Bacon,  Dench,  and  many  aural  surgeons. 

Abscess  of  the  brain  in  connection  with  diseases  of  the  nose  must  be 
explained  in  much  the  same  way  (Dreyfuss).  The  nasal  disease  is  sometimes 
limited  to  the  mucous  membrane  without  any  involvement  of  the  bone,  yet 
abscesses  form  just  the  same.  Under  such  conditions  the  toxic  substance 
must  be  carried  along  the  blood-vessels  or  the  lymphatics.  If  the  nasal 
bones  are  the  seat  of  suppuration,  as  in  syphilis,  the  abscess  that  forms  may 
be  in  direct  connection  with  the  nasal  cavity,  and  the  pus  from  the  brain  may 
be  freely  discharged  through  the  nose. 

The  breaking  down  of  tubercular  growths  may  occasionally  be  the  cause 
of  abscess,  but  these  cases  have  less  practical  interest,  for  the  general  tuber- 
cular character  of  the  disease  is  the  significant  feature,  and  the  abscess,  as  a 
rule,  a  mere  incident  in  the  course  of  a  protracted  illness. 

Purulent  disease  in  any  other  part  of  the  body  may  also  be  the  cause  of 
abscess  of  the  brain  through  the  agency  of  a  septic  embolus.  Gowers  quotes 
Boettcher's  report  of  a  case  in  which  a  cerebral  abscess  secondary  to  suppura- 
tion in  the  lung  was  found  to  contain  lung  pigment.  There  is  a  remarkable 
connection  between  brain  abscess  and  every  form  of  purulent  disease  within 
the  thoracic  cavity.  Thus  we  have  cases  of  brain  abscess  after  pneumonia 
in  which  the  exudate  has  not  been  perfectly  resolved  and  has  undergone  sup- 
puration. Pus  cavities  occur  after  purulent  bronchitis  and  empyema ;  re- 
cently Moschowitz  operated  upon  a  young  boy  in  whom  the  author  had 
helped  to  locate  an  abscess  in  the  Rolandic  area  following  empyema. 

As  a  rare  cause  of  abscess,  but  one  that  is  worth  mentioning,  we  may  note 
its  occurrence  after  thrush  in  two  cases  reported  by  Zenker.  One  patient  was 
an  infant.     In  these  cases  the  brain  was  studded  with  small  abscesses  con- 


ABSCESS   OF  THE   BRAIN.  49 1 

taining  the  fungus  of  thrush  (oi'dium  albicans;  ;  the  fungus  had  evidently 
travelled  by  way  of  the  blood-vessels,  since  it  had  actually  been  demonstrated 
in  their  interior. 

Symptomatology. — Few  conditions  are  more  difficult  to 
recognize  during:  life  than  abscesses  of  the  brain.  It  is  safe 
to  say  that  they  constitute  a  large  proportion  of  the  sur- 
prises and  disappointments  of  the  post-mortem  table.  They 
are  found  when  least  expected,  and  when  confidently  looked 
for  are  often  wanting.  The  uncertainty  in  the  diagnosis  is 
due  to  the  very  insidious  development  of  the  process  and 
to  the  fact  that  when  once  encapsulated  a  pus  cavity  gives 
rise  to  very  few  symptoms. 

When  fully  developed  an  abscess  practically  constitutes 
a  foreign  body  in  the  brain ;  we  would  therefore  expect 
such  symptoms  as  are  commonly  due  to  tumors  within  the 
brain  or  cranial  cavity,  but  in  this  we  are  apt  to  be  disap- 
pointed, for  the  brain  appears  to  accommodate  itself  to  the 
slowly  increasing  abscess  and  does  not  respond  to  this 
growth  as  distinctly  as  it  does  to  the  invasion  of  a  solid 
neoplasm.  The  abscesses  do  not,  moreover,  occur  as  fre- 
quently as  tumors  do  in  those  parts  of  the  brain  which  give 
rise  to  signal  symptoms  (the  motor  area  and  the  occipital 
lobes).  Abscesses  are  more  common  in  the  frontal  lobes,  in 
the  temporo-sphenoidal  lobes,  and  in  the  cerebellum.  In 
acute  cases,  favorable  for  diagnosis,  the  first  symptoms  are 
those  with  which  we  are  familiar  in  connection  with  menin- 
geal disturbances,  such  as  local  painfulness  of  the  scalp, 
nausea,  vomiting,  vertigo,  and  fever.  The  last,  if  irregu- 
lar and  if  attended  by  rigors,  is  the  one  symptom  which 
suggests  abscess  much  more  forcibly  than  any  other.  If 
the  cavity  is  in  the  vicinity  of  the  motor  areas  and  is  not 
too  rapidly  destructive,  localized  convulsions  may  occur. 
Abscess  in  this  region,  which  is  supplied  by  the  middle 
cerebral  artery,  is  very  likely  to  be  due  to  septic  embolism  ; 
hence  the  formation  of  pus  in  this  region  after  lung  disease 
and  after  septic  endocarditis.  Paralysis  may  be  present  in 
some  of  these  cases  if  the  motor  area  is  involved,  but  if  the 
abscess  is  elsewhere  in  the  brain  paralytic  symptoms  will 
not  constitute  a  prominent  feature  of  the  disease  until  the 
terminal  stage  has  been  reached. 


492  THE  NERVOUS  DISEASES   OF  CHILDREN. 

To  this  category  of  symptoms,  delirium,  convulsions,  ir- 
regular or  high  temperatures  may  be  added,  which  lead  to 
a  rapidly  fatal  issue. 

Chronic  abscess  may  exist  for  a  number  of  years  and 
may  give  rise  to  very  few  symptoms  ;  in  one  case  of  chronic 
abscess,  in  a  boy  of  eight  years,  no  s}^mptoms  were  present 
as  long  as  the  ear  freely  discharged.  As  soon  as  this 
ceased,  epileptiform  convulsions,  preceded  by  sensory  aurae 
(disagreeable  odor  and  a  hissing  noise)  would  occur.  This 
alternation  was  kept  up  for  at  least  two  years,  when  the 
boy  died  of  a  general  purulent  meningitis  from  bursting  of 
the  abscess  into  the  temporo-sphenoidal  lobe.  The  so- 
called  latent  state  of  the  abscess  is  characterized,  as  a  rule, 
by  occasional  spells  of  nausea,  by  occasional  vomiting,  and 
by  intermittent  febrile  attacks  for  which  no  proper  expla- 
nation can  be  found  at  the  time.  Persistent  headaches,  if 
associated  with  ear  disease  or  with  other  conditions  that 
give  rise  to  abscess,  point  to  the  existence  of  this  condition. 
The  headache  is  generally  more  or  less  circumscribed,  and 
sometimes  varies  according  to  the  position  of  the  head  ;  but 
this  mere  fact  of  change  of  position  as  affecting  the  head- 
ache does  not  necessarily  point,  as  one  would  suppose,  to 
abscess,  for  I  have  known  the  same  condition  to  accompany 
solid  tumor  of  the  brain.  The  pain,  as  a  rule,  is  referred 
to  the  vicinity  of  the  abscess,  but  not  invariably  so.  Mac- 
ewen's  symptom  (as  described  in  Chapter  XXVI.)  may  be 
of  service,  but  should  be  utilized  with  great  caution.  The 
temperature  is  often  lowered  in  abscess  of  the  brain,  and  is 
accompanied  by  slow  pulse.     (Macewen.) 

Optic  neuritis,  so  characteristic  of  tumor,  also  occurs  in 
abscess  of  the  brain.  It  is  not  as  constant  a  symptom  in  the 
latter  as  in  the  former  condition  ;  according  to  Okada's  sta- 
tistics (quoted  by  Oppenheim)  optic  neuritis  occurred  in 
two-thirds  of  the  cases.  The  neuritis  is  in  nowise  to  be  dis- 
tinguished from  the  neuritis  associated  with  ordinary  tu- 
mors within  the  cranial  cavity.  The  other  symptoms  will 
vary  very  much  according  to  the  location  of  the  abscess. 
Paralysis,  if  present,  is  apt  to  be  hemiplegic.  If  associated 
with  unilateral  convulsions  it  points  to  the  motor  cortex  as 
the  site  of  the  abscess.     Sensation  is,  as  a  rule,  not  involved, 


ABSCESS    OF   THE   BRAIN.  493 

and  the  various  cranial  nerves  are  not  affected  unless  the 
abscess  is  at  or  near  the  base.  The  fifth  and  sixth  nerves 
may  be  pressed  upon  by  a  cerebellar  abscess,  causing  pain 
in  the  face  and  paralysis  of  the  muscles  of  mastication 
and  of  the  rectus  externus.  Cerebral  abscess  may  also  be 
associated  with  paralysis  of  the  facial  nerve,  but  this  is  only 
rarely  due  to  involvement  of  the  nerve  as  it  emerges  from 
the  pons,  but  is  more  frequently  of  old  standing  and  due  to 
an  early  disease  of  the  ear  affecting  the  nerve  in  its  course 
through  the  Fallopian  canal.  Paraphasia,  amnesic  aphasia, 
alexia,  agraphia,  accompany  abscess  involving  parts  con- 
nected with  the  function  of  speech.  In  several  patients 
whom  I  had  the  privilege  of  seeing  with  Gruening,  sensory 
aphasia  was  the  first  signal  symptom  of  an  abscess  in  the 
temporo-sphenoidal  lobe.  Hemianopsia  may  be  present 
(case  of  Knapp).  Stupor,  delirium,  and  coma  mark  the  ter- 
minal stages  of  abscess.  In  cerebellar  abscess  the  charac- 
teristic signs  are:  Pain  over  the  occiput  and  the  region  of 
the  neck;  rigidity  of  the  neck;  vertigo,  inco-ordination  and 
typical  cerebellar  ataxia ;  vertigo  and  vomiting  when  the 
position  of  the  head  is  changed  (Bruns);  bulbar  symptoms. 
Differential  Diagnosis. — From  the  account  of  the 
symptoms  accompanying  abscess  of  the  brain  it  is  evident 
that  it ,  is  difficult,  sometimes  impossible,  to  differentiate 
between  a  pus  cavity  in  the  brain  and  other  conditions. 
Among  these  are  solid  tumor  of  the  brain,  meningitis,  and 
meningo-encephalitis.  The  diagnosis  of  solid  tumor  can 
more  readily  be  made  if  the  usual  causes  for  abscess,  such 
as  ear  disease  and  external  injury,  are  wanting,  and  if  the 
symptoms  have  developed  in  a  subacute  fashion,  which 
is  rather  characteristic  of  tumor,  while  in  abscess  there  is 
either  a  prolonged  latent  period  or  all  the  symptoms  de- 
velop in  a  very  short  period  of  time.  Suspicion  of  abscess 
is  strengthened  above  all  things  by  the  presence  of  marked 
rigors  and  rapidly  changing  temperatures.  A  slight  change 
of  temperature  may  occur  in  the  case  of  tumors,  but  it  is 
not  as  variable  and  does  not  show  the  extremes  so  common 
in  abscess.  During  the  past  years  I  have  observed  several 
cases  of  abscess  in  which  there  was  not  the  slightest  rise  of 
temperature  for  several  weeks  preceding  death. 


494  THE  NERVOUS  DISEASES   OF  CHILDREN. 

The  differential  diagnosis  between  tumor  and  abscess  is  one  of  practical 
importance.  If  the  new-growth  is  near  the  surface,  and  if  the  symptoms  are 
of  such  a  kind  as  to  lead  one  to  infer  a  tumor  of  considerable  extent,  the 
surgeon  might  well  hesitate  to  operate  if  he  thought  the  new-growth  a  solid 
one.  If  there  is  a  probability  of  an  abscess  the  size  of  the  abscess  need  not 
constitute  a  contraindication  to  operation,  and  this  differentiation  between 
tumor  and  abscess  is  still  more  important  if  the  growth  is  supposed  to  be  in 
the  interior  of  the  brain.  If  this  should  be  a  solid  tumor  a  surgical  opera- 
tion would  do  no  good,  but  if  it  is  an  abscess  it  could  well  be  drained  through 
the  hemispheres,  and  the  greatest  danger  of  all,  rupture  into  the  ventricles, 
might  be  averted. 

The  differentiation  between  meningitis  and  abscess  is  not  an  easy  one, 
particularly  in  cases  of  ear  disease  and  external  injuries.  Meningitis  is  also 
apt  to  be  associated  with,  or  to  precede,  the  formation  of  abscess,  and  during 
this  period  the  question  whether  the  suppurative,  and  hence  the  operative, 
stage  has  been  reached  is  not  always  easy  to  decide.  Moreover,  in  the  final 
stages  of  abscess,  particularly  if  the  abscess  has  ruptured,  a  general  purulent 
meningitis  is  the  invariable  result.  At  that  stage  a  differential  diagnosis  can 
easily  be  made  with  a  great  degree  of  certainty,  but  if  made  has  little  practical 
value.  In  the  earlier  stages  of  cerebral  disease  after  otitis  or  external  injuries 
it  is  safe  to  believe  that  the  condition  is  still  one  of  meningitis,  without  forma- 
tion of  abscess,  if  the  symptoms  are  those  of  a  general  cerebral  disturbance, 
such  as  vertigo,  headaches,  nausea,  slight  stupor,  but  without  rigors  or  serious 
rise  of  temperature.     Localizing  symptoms  may  be  present  in  both  cases. 

The  distinction  between  meningitis  and  abscess  is  of  much  practical  im- 
portance for  another  reason.  The  majority  of  surgeons  would  not  be  willing 
to  operate  upon  cases  of  meningitis  ;  they  evidently  have  the  general  puru- 
lent meningitis  in  mind ;  with  improved  methods  in  surgery,  exploratory 
trephining  may  be  warranted,  particularly  in  connection  with  ear  disease  or 
external  injury  if  there  is  a  suspicion  of  meningitis  alone,  provided  the  latter 
be  limited  in  extent.     Lumbar  puncture  may  give  diagnostic  aid. 

In  practice  the  differential  diagnosis  most  frequently  to  be  established  is 
between  abscess  and  sinus  thrombosis  after  middle-ear  disease.  The  gen- 
eral cerebral  symptoms  may  be  similar  in  both  conditions,  but  the  special 
symptoms  of  sinus  thrombosis  (see  p.  498)  will  help  to  decide  the  point.  In 
many  cases  the  condition  is  uncertain  before  the  operation  is  undertaken. 
In  every  doubtful  case  the  surgeon  should  look  first  for  sinus  thrombosis, 
and  then  for  abscess. 

Prognosis. — The  prognosis  of  abscess  of  the  brain  is  in- 
variably grave.  While  admitting  the  possibility  of  an  ab- 
cess  becoming  encapsulated  and  of  remaining  latent  in  the 
brain  for  many  years,  the  probability  is  in  every  instance 
that  serious  mischief  will  result  from  it  sooner  or  later. 
The  physician  must  not  trust  to  good  fortune  in  such  dis- 
eases, but  knowing  the  serious  nature  of  the  trouble  he  is 


ABSCESS   OF   THE  BRAIN.  495 

bound  to  present  to  the  patient,  or  the  patient's  relatives, 
the  alternatives  of  surgical  interference  or  death  within  a 
limited  period  of  time.  The  longer  the  abscess  has  lasted 
the  more  serious  it  is  apt  to  be,  for  the  danger  of  rupture 
becomes  greater  with  the  increase  in  its  size. 

Treatment. — In  spite  of  recent  advances  in  cranial  sur- 
gery and  of  the  successes  that  have  been  recorded  in  the  treat- 
ment of  abscess  of  the  brain  it  is  best  to  discuss  the  proph- 
ylactic measures  which  may  possibly  prevent  the  formation 
of  abscess.  Knowing  that  pus  is  formed  most  frequently 
after  ear  disease  and  after  disease  of  the  bone,  the  greatest 
caution  should  be  exercised  to  prevent  any  extension  of  the 
inflammatory  process  into  the  cranial  cavity  from  these 
original  sources  of  infection.  The  possibility  of  cerebral 
complications  in  every  case  of  suppurative  ear  disease 
should  be  kept  well  in  mind,  and  free  discharge  outward 
should  be  secured  and  maintained.  In  the  case  of  mastoid 
and  middle-ear  disease  we  are  not  only  bound  to  secure  a 
free  passage  outward  for  the  pus,  but  the  discharge  should 
be  watched,  and  as  soon  as  the  pus  ceases  to  flow  outward 
while  the  symptoms  point  to  a  constant  generation  of  pus 
in  the  bony  structures,  surgical  measures  should  be  resorted 
to  to  drain  away  the  pus  that  is  formed.  While  pus  is  dis- 
charging freely  from  the  internal  or  the  middle  ear,  if  symp- 
toms arise  pointing  to  meningeal  or  cerebral  involvement, 
such  as  increasing  headaches,  vertigo,  vomiting,  and  fever, 
further  surgical  aid  is  needed. 

An  attempt  may  be  made  when  the  first  meningeal 
symptoms  arise  to  treat  these  in  the  ordinary  way  by  the 
application  of  cold,  by  blistering,  by  counter -irritation, 
and  the  application  of  leeches  and  the  like,  but  very  little 
time  should  be  wasted  with  such  measures,  and  if  the 
symptoms  do  not  yield  within  twenty-four  or  forty-eight 
hours  the  surgeon  must  afford  the  needed  relief.  In  the 
case  of  abscess  due  to  ear  disease,  until  recently  little  suc- 
cess has  attended  the  efforts  of  the  surgeon  to  locate  and 
drain  the  abscess;  but  it  bids  fair  to  become  the  most  satis- 
factory and  legitimate  cause  for  operation  upon  the  brain. 
This  opinion  (of  the  first  edition)  has  received  fullest  indorse- 
ment by  the  experience  of  recent  years. 


496 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


The  temporal  convolutions  can  be  exposed  by  trephining 
at  a  point  which  is  reached  by  drawing  a  line  one  and  a 
quarter  inch  back  from  the  external  auditory  meatus,  and 
drawing  another,  at  right  angles  from  this  one,  the  same 
distance  upward.  The  terminating  point  of  this  line  may 
be  made  the  centre  of  the  trephine  opening,  and  will  be 
sufficiently  accurate  to  enable  the  surgeon,  after  removing 
a  considerable  portion  of  the  bone,  to  expose  the  region 
of  the  abscess.  As  the  abscess  is  not  always  superficial, 
punctures  should  be  made  with  a  fine  hypodermic  needle  in 
various  directions  in  order  to  reach  cavities  that  are  situated 

below  the  gray  mat- 
ter. By  observing 
these  rules  one  can 
hardly  fail  to  find  the 
abscess.  In  one  case 
of  the  author's,  the 
surgeon  had  to  make 
four  punctures  into 
the  substance  of  the 
brain  before  pus  was 
withdrawn.  Absces- 
ses formed  in  connec- 
tion with  disease  of 
other  parts  of  the  cra- 
nium can  generally  be  found  if  one  is  guided  by  the  external 
signs ;  thus,  in  a  case  which  was  operated  on  for  me  nearly 
ten  years  ago,  there  was  caries  in  the  middle  of  the  parietal 
bone.  The  surgeon  was  directed  to  open  the  skull  at  this 
point,  and,  if  no  abscess  presented,  to  insert  the  needle  in 
various  directions.  When  the  dura  was  exposed  nothing  ab- 
normal was  noticed,  but  the  first  thrust  of  the  needle  secured 
a  large  quantity  of  pus ;  the  brain  tissue  was  then  incised 
and  the  entire  abscess  cavity  opened,  but  the  patient  died  a 
few  days  later,  and  on  the  post-mortem  table  other  abscesses 
were  found  in  the  opposite  hemisphere  of  the  brain  as  well 
as  at  the  base.  If  multiple  abscesses  can  be  diagnosticated, 
operative  interference  is  useless.  In  cases  of  skull  injury 
the  trephine  opening  should  always  be  made  at  the  seat  of 
the  external  injur}',  and  enlarged  from  this  point  to  meet 


■^-p-f  "•"  ***' 


TttJ. 


Fig.  123 — Dissections  showing  the  Guide  Adopted 
by  Barker  in  Successful  Trephining  for  Abscess 
from  Ear  Disease.     (After  Gowers.) 


ABSCESS   OF   THE   BRAIN.  497 

the  exigencies  of  a  given  case.  If  the  pus  cavity  be  due  to 
disease  of  the  nose,  a  large  opening  in  the  frontal  bone 
should  be  made,  from  which  the  surgeon  will  then  attempt 
to  locate  the  exact  seat  of  the  abscess,  and,  if  possible,  drain 
it  according  to  the  usual  method. 

Abscesses  in  the  cerebellum  are  now  within  reach  of 
the  surgeon's  knife.  Koerner  has  collected  55  cases  of 
cerebellar  abscess,  with  a  favorable  result  in  52.8  per  cent, 
of  the  cases.  It  is  well,  however,  to  remember  that  the 
majority  of  unsuccessful  cases  are  not  recorded. 

Successful  treatment  of  brain  abscess  has  been  reported  by  Stimson, 
Bergmann,  Schede,  Paget,  Park,  Koerte,  Jansen,  Knapp,  Gruening,  Starr, 
Saenger  and  a  host  of  others.  The  case  of  Knapp  is  of  special  interest,  as 
it  occurred  in  a  child  nine  years  of  age.  The  chief  symptoms  were  chronic 
otorrhcea  (after  scarlet  fever),  optic  neuritis,  homonymous  hemianopsia,  and 
Macewen's  symptom  (percussion  note  stronger  on  left  side) ;  there  was  no 
sensitiveness  on  percussion  of  the  skull.  At  the  operation  a  pus  cavity  was 
found,  and  complete  recovery  ensued  in  the  course  of  several  weeks,  after 
minor  mishaps. 

There  is  no  reason  why  children  who  present  indubitable 
signs  of  brain  abscess  should  not  be  operated  upon  according 
to  the  same  principles  which  hold  good  in  adults.*  It  is 
probable  that  with  greater  experience  in  these  cases  the 
operation  need  not  last  several  hours,  as  in  some  of  those 
hitherto  reported,  and  hernia  cerebri  can  surely  be  avoided. 

THROMBOSIS   OF   THE    INTRA-CRANIAL    SINUSES. 

The  blood  circulates  in  the  venous  sinuses  within  the  skull  under  special 
disadvantages.  The  walls  of  the  sinuses  are  rigid,  the  blood  cannot  be  emp- 
tied during  inspiration,  and  many  of  the  cerebral  veins  join  the  longitudinal 
sinus  at  an  obtuse  or  right  angle,  so  that  their  blood  is  poured  into  the 
superior  sinus  in  a  direction  opposite  to  the  main  current.  Under  such  con- 
ditions it  is  natural  that  the  current  should  be  retarded  and  coagulation  of 
the  blood  easily  set  in. 

Two  forms  of  thrombosis  occur ;  the  first  is  the  primary  or  marasmic 
thrombosis  which  occurs  in  children  after  exhausting  diseases  (cholera  infan- 
tum, etc.).  This  is  generally  confined  to  the  longitudinal  sinus,  but  may 
involve  the  lateral  and  cavernous  sinuses.  In  such  cases  the  clots  are  dense, 
resistant,  organized,  and  do  not  adhere  to  the  wall  of  the  vein.     The  throm- 

*  The  readiness  of  the  aural  surgeons  to  open  up  the  mastoid  will  do  much  to 
diminish  the  frequency  of  brain  abscess. 


498 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


bosis  may  be  limited  to  the  cerebral  veins,  thus  giving  rise  to  limited  or  cor- 
tical symptoms.  Gowers,  it  will  be  remembered,  has  attached  special  signifi- 
cance to  this  condition  in  the  causation  of  infantile  cerebral  palsies. 

The  second  form  is  the  secondary  or  infective  thrombosis,  which  generally 
affects  the  lateral,  the  cavernous,  or  the  transverse  sinuses.  It  is  secondary 
to  some  infective  process  in  the  neighboring  tissues  or  at  a  distance.  Middle- 
ear  disease  is  the  most  frequent  cause,  but  it  may  also  be  due  to  some  trau- 
matism of  the  skull  (infected  wounds),  erysipelas  of  the  head  and  face,  to 
purulent  disease  of  the  eyes  and  of  the  nose.  In  one  of  the  author's  cases 
caries  of  the  jaw  was  the  starting-point  of  sinus  thrombosis.      In  other 


Fig.  124. — Girl,  Aged  Twenty.  Exophthalmus,  with  internal  strabismus  ;  oedema  of 
the  right  eyelids,  side  of  the  nose,  the  brow,  and  the  face.  Later  in  the  disease  the 
left  side  was  also  affected.     Thrombosis  of  cavernous  sinus.     (Macewen  ) 


cases  the  infectious  material  is  carried  along  the  veins  and  through  neighbor- 
ing tissues. 

The  inflammation  may  extend  directly  to  the  walls  of  the  sinus  and  thus 
cause  clotting  of  the  blood  within,  or  the  clot  may  form  within  a  vein  and 
extend  from  there  into  the  sinus.  The  superior  petrosal  and  the  lateral 
sinuses  receive  their  blood  from  the  middle  ear,  hence  the  frequency  of  throm- 
bosis of  these  sinuses  in  middle-ear  disease.  Thrombosis  of  the  sinus  result- 
ing from  actual  compression  is  very  rare  indeed  and  of  little  significance,  as 
the  symptoms  would  be  obscured  by  those  of  the  primary  affection. 

Symptoms. — The  symptoms  of  sinus  thrombosis  are  often  complicated 
by  those  of  the  primary  disease.  We  must  distinguish  between  general  and 
special  symptoms.  Among  the  former  manifestations  the  most  important  are 
intense  headaches,  somnolence  increasing  to  stupor  and  coma,  convulsions, 
slight  rigidity  of  the  neck,  optic  neuritis,  rigors,  accelerated  or  diminished 


ABSCESS   OF   THE    UK  A  IN.  4</j 

pulse-rate,  thready  pulse,  and  fluctuating  temperatures ;  but  most  of 
symptoms  would  suggest  meningitis  or  encephalitis  quite  as  readily,  if  the 
conditions  favoring  sinus  thrombosis  were  not  known  to  be  present ;  more- 
over, actual  meningitis  may  be  caused  by  thrombosis  of  the  sinus,  particu- 
larly if  such  thrombosis  is  of  an  infective  nature. 

The  special  symptoms  refer  particularly  to  disturbances  in  the  venous 
circulation  and  to  areas  of  tenderness.  Thrombosis  of  the  superior  longi- 
tudinal sinus  is  characterized  by  oedema  of  the  scalp  and  distention  of  the 
veins  in  the  parietal,  frontal,  and  occipital  regions.  In  infants  the  anterior 
fontanelle  becomes  prominent,  and  epistaxis  is  frequent ;  squint  is  a  rare 
occurrence.  Cavernous  sinus  thrombosis  is  rare  (the  author  has  seen  two 
cases  within  a  single  year)  ;  the  thrombosis  is  generally  due  to  extension 
from  other  sinuses,  from  the  petrosal  or  lateral  sinus,  or  it  may  result  from 
disease  of  the  ophthalmic  veins  (Knapp)  and  abscess  in  the  orbital  cavity. 
In  more  than  half  of  the  cases  the  thrombosis  spreads  to  the  cavernous  sinus 
of  the  opposite  side.  The  special  symptoms  are  exophthalmus,  oedema  of 
the  eyelids  and  root  of  the  nose  due  to  venous  obstruction  ;  also  ptosis,  paral- 
ysis of  rectus  externus,  and  supraorbital  pain,  all  due  to  compression  of  various 
nerves  to  the  orbit  running  close  to  the  sinus.  (See  Fig.  124a.)  "Choked 
disk "  from  obstruction  of  the  retinal  and  ophthalmic  vessels  is  present  in 
some  cases  of  cavernous  sinus  thrombosis.  Defect  of  vision  and  amaurosis 
may  be  superadded.  Some  of  these  cases  live  five  or  nine  months,  but  all 
end  fatally.  The  author  had  a  case  under  observation  in  which  the  condition 
has  remained  practically  unchanged  for  nearly  two  months. 

Thromboses  of  the  petrosal  and  lateral  sinuses  are  the  most  frequent,  and 
the  most  important ;  unfortunately  they  present  very  few  special  symptoms. 
In  lateral  sinus  thrombosis  there  may  be  distention  of  veins  and  oedema  over 
the  mastoid  process,  and  tenderness  on  percussion  in  this  region,  but  the 
symptoms  differ  very  little  from  those  due  to  ear  disease  and  localized  men- 
ingitis, and  all  three  conditions  may  be  present.  Inflammation  of  the  veins 
leading  from  the  lateral  sinus  occurs  in  cases  of  infective  thrombosis.  When 
these  veins  are  inflamed  pain  may  be  elicited  along  the  internal  jugular  vein, 
and  over  the  upper  third  of  the  posterior  cervical  triangle.  A  difference  in 
the  sensitiveness  of  the  two  sides  will  be  strong  corroborative  evidence  of 
suspected  thrombosis.  Cervical  abscess  and  enlargement  of  cervical  glands 
may  result  from  infective  external  thrombosis  of  the  lateral  sinus. 

The  prognosis  of  sinus  thrombosis  is  always  grave.  A  fatal  issue  is  to 
be  expected,  although  death  may  be  delayed  for  a  period  of  several  months. 
The  only  hope  lies  in  successful  surgical  interference  and  in  early  preventive 
measures.  A  few  recoveries  are  on  record.  The  lateral  and  transverse 
sinuses  have  been  opened  successfully  and  the  clots  removed.*  Ballance, 
Sulzer,  Schwartze,  Parkin,  Horsley,  Macewen,  Bacon,  Pritchard,  and  Cheadle 
have  reported  successful  cases.  Parkin's  and  Cheadle's  patients  were  respect- 
ively nine  and  thirteen  years  of  age.     Henschen  has  collected  145  cases  of 

*  The  details  of  the  operation  should  be  studied  in  Macewen's  treatise,  page  307 
et  seq.  j 


5oo 


THE  NERVOUS  DISEASES   OF  CHILDREN. 


operation  for  sinus  phlebitis  following  otitis  ;  of  these,  85  were  cured.  Tying 
the  jugular  vein  before  removing  the  clot  seems  to  exert  a  favorable  influence 
upon  the  percentage  of  cures.  Koerner  states  that  there  was  recovery  in 
seventy-five  per  cent,  of  the  cases  if  the  jugular  vein  was  tied,  and  only  fifty 
per  cent,  recovered  if  the  vein  was  not  tied.  Jansen  does  not  favor  this 
procedure  in  all  cases. 


,,  c'onfluens   sintium  I 
s.Torcular  UerophUi 


Fig.  124a. — The  Veins  of  the  Dura  Mater.     (Heitzman. ) 


The  extensive  experience  of  aurists  and  a  far  larger  collection  of  cases 
have  helped  to  decide  many  mooted  points,  and  the  successful  operations 
recorded  encourage  one  to  hope  that  sinus  thrombosis  (excepting  possibly  as 
part  of  a  general  pyaemia)  will  become  an  eminently  curable  disease.  Explora- 
tory operations  in  doubtful  cases  are  not  only  justifiable  but  imperatively 
demanded. 


CHAPTER   XXVIII. 

DISEASES    AND    CONDITIONS    DUE    TO    DEFECTIVE    DEVEL- 
OPMENT  OF   THE   BRAIN. 


LARGE   DEFECTS. 

Just  as  there  are  abnormalities  of  the  spinal  cord,  due  to  defective  closure 
of  the  vertebral  arches,  so  there  are  deformities  of  the  brain  and  its  coverings 
due  to  defective  closure  of  the  skull.  The  various  forms  of  protrusion  are 
best  illustrated  by  Figs.  125-127,  taken  from  HoU's  Textbook.     These  de- 


FiG.  125. — Meningocele. 


Fig.  126. — Encephalocele. 
(After  Holt.) 


Fig.  127. — Hydrencephalocele. 


formities  are  rare  and  of  little  interest,  although  Treves  and  others  have 
attempted  to  remedy  these  conditions  by  surgical  means.  Most  of  the  chil- 
dren thus  afflicted  die  within  a  few  weeks. 

In  meningocele,  the  membranes  only  are  protruded;  in  encephalocele 
some  brain  substance  is  protruded  ;  in  hydrencephalocele  the  protruding  brain 
substance  contains  a  cavity  which  is  in  direct  communication  with  the  dis- 
tended lateral  ventricle.  These  tumors,  varying  much  in  size,  may  be  occip- 
ital or  frontal ;  they  may  be  covered  by  the  scalp,  or  only  by  granulation  tis- 
sue ;  other  deformities  are  often  associated  with  them. 

Cyclops  or  monopus,  as  the  name  tells,  indicates  a  condition  in  which 
but  one  orbit  exists  in  the  middle  of  the  face,  and  about  in  the  position  gen- 
erally occupied  by  the  root  of  the  nose.  The  size  of  this  one  orbital  cavity 
varies  according  to  the  development  of  the  eye.  In  some  cases  the  eye  is 
not  present,  and  the  orbital  cavity  is  correspondingly  small.  The  cavity  at- 
tains its  largest  size  if  the  two  eyes  are  fused  into  one;  and  in  accordance 
with  the  single  or  double  development  of  the  eye,  one  or  two  optic  nerves  may 
be  present.  If  no  bulb  has  been  developed  there  is  also  a  corresponding  de- 
fect of  the  optic  nerve.     The  eyelids  may  be  single  or  double,  the  nose,  as  a 


502  THE   NERVOUS  DISEASES    OF    CHILDREN. 

rule,  is  undeveloped,  and  its  place  indicated  by  something  which  simulates 
a  short  snout.  The  entire  features  are  distorted,  the  mouth  may  be  wanting, 
and  the  ears  come  closely  together. 

The  only  reason  that  we  have  to  consider  this  condition  at  all  is  that  it  is 
due  to  a  defective  development  of  the  anterior  cerebral  vesicle  ;  the  ganglia, 
too,  are  more  or  less  deficient ;  the  corpus  callosum  may  be  wanting  alto- 
gether. The  parts  of  the  brain  coming  from  the  middle  and  hind  brain  vesi- 
cle are,  as  a  rule,  better  developed  than  the  hemispheres  are. 

Fortunately  these  cases  are  not  viable ;  they  have  therefore  no  clinical 
value  whatever,  and  may  well  be  relegated  to  the  authors  specially  interested 
in  monstrosities. 

The  next  most  frequent  and  most  complete  anomaly  of  brain  structure 
is  the  condition  known  as  anencephalus  or  hemicephalus.  The  difference 
existing  between  the  two  conditions  consists,  as  the  names  indicate,  in  the 
complete  or  partial  absence  of  brain  development.  Steffen  assumes,  without 
decided  evidence,  that  these  departures  from  normal  development  are  due  to 
an  inflammation  of  the  meninges  occurring  during  the  fcetal  period,  in  conse- 
quence of  which  a  large  amount  of  cerebro-spinal  fluid  collects  in  the  ventri- 
cles, in  the  meshes  of  the  pia,  and  in  the  subdural  spaces ;  and  that  in  conse- 
quence of  this  increased  pressure  upon  the  developing  parts  the  normal 
growth  of  the  brain  is  interfered  with.  There  is  no  reason  to  dispute  this 
view  until  some  better  opinion  is  offered.  The  only  suggestion  to  be  made  is 
that  the  inflammation  may  have  been  preceded  by  disturbances  of  circulation, 
and  if  such  disturbance  of  circulation  is  the  important  factor,  we  shall  have 
the  same  cause  operative  in  these  cases  of  larger  defects  as  in  those  smaller 
defects  called  porencephaly,  which  we  shall  study  hereafter.  In  cases  of  an- 
encephaly  or  hemicephaly  a  very  considerable  portion  of  the  skull  may  be  ab- 
sent. There  may  be  rudiments  of  the  parietal,  of  the  temporal,  or  of  the 
occipital  bones.  In  some  cases  a  portion  of  the  occipital  bone  is  left,  and 
this,  together  with  the  petrous  portion  of  the  temporal  bone,  provides  a  small 
groove  in  which  some  cerebellar  rudiments  may  be  lodged.  The  frontal  bone 
is,  as  a  rule,  undeveloped.  If  present,  it  is  very  low,  the  eyes  therefore  pro- 
trude, the  face  points  upward,  and  the  jaws  push  forward.  Instead  of  the 
roof  of  the  skull,  a  thickened  membrane  may  be  all  that  is  present.  In  other 
cases  the  bony  defect  may  be  covered  by  a  tolerably  normal  scalp,  which 
may  be  fused  with  the  dura  mater. 

If  the  skull  is  opened  every  conceivable  defect  may  be  found.  In  the  ma- 
jority of  cases  after  removal  of  the  bone,  the  thickened  membranes  present 
themselves,  and  if  these  are  punctured  a  very  large  amount  of  fluid  flows  out, 
the  membranes  collapse,  and  further  examination  of  the  specimen  may  show 
nothing  excepting  the  basilar  structures,  no  trace  of  the  hemispheres  or 
any  part  of  the  frontal  brain  being  present. 

PORENCEPHALY. 

The  condition  of  porencephaly  is  much  more  interesting  from  every  point 
of  view  than  the  larger  brain  defects  previously  mentioned.     The  term  por- 


DEFECTIVE  DEVELOPMENT  OF   THE    BRAIN,  503 

encephaly  has  been  much  abused.  It  has  been  made  to  embrace  an  entire 
absence  of  brain  structure,  or  very  minute  defects  in  the  brain,  secondary  to 
vascular  lesions.  It  would  be  best  to  use  the  term  in  the  way  it  was  first 
used  by  Heschl,  who  invented  it,  and  by  Kundrat,  who  wrote  the  first  exten- 
sive monograph  on  the  subject.  As  the  name  indicates,  it  is  practically  a 
hole  in  the  brain,  not  an  entire  absence  of  brain  structure.  For  that  reason 
there  should  be  some  evidence  of  development  of  that  part  of  the  brain  in 
which  the  hole  lies.  This  hole,  or  porus,  may  be  the  result  of  defective  de- 
velopment, or  it  maybe  acquired  in  the  latest  period  of  fcetal  life,  or  even  in 
the  first  period  of  life  after  birth.  It  would  be  well  to  distinguish  the  truly 
congenital  cases  from  the  acquired  cases,  with  the  additional  qualification 
that  even  the  truly  congenital  cases  may  be  acquired  in  the  sense  of  being 
the  result  of  some  morbid  process  during  the  fcetal  period. 


Congenital  Porencephaly. — The  defect  may  be  in  the 
anterior,  in  the  middle,  the  posterior,  .or,  in  fact,  in  any 
part  of  the  brain,  but  it  is  found  much  more  frequently  in 
the  anterior  and  in  the  middle  portion.  Congenital  poren- 
cephaly may  be  single  or  double. 


An  extreme  condition  of  porencephaly  will  be  best  illustrated  by  Figs.  128 
and  129,  taken  from  Schultze's  monograph,  which  he  entitles  "  A  Contribution 
to  the  Doctrine  of  Congenital  Cerebral  Defects."  I  select  this  case  because  it 
represents,  as  it  seems  to  me,  an  extreme  condition  of  porencephaly,  and  is 
only  one  stage  short  of  a  defective  development  involving  a  very  large  portion 
of  the  entire  brain.  By  reference  to  the  figures  it  will  be  seen  that  the  posterior 
half  of  the  brain  is  tolerably  well  developed,  but  that  the  anterior  half  is 
practically  nothing  but  a  sac  enclosed  by  a  thick  membrane.  The  defect  in- 
cluded not  only  the  two  frontal  and  a  considerable  portion  of  both  parietal 
lobes,  but  the  greater  part  also  of  both  temporal  lobes.  The  posterior  half  of 
the  brain,  the  cerebellum,  and  all  the  ganglia  proved  normal  on  microscopical 
examination.  Schultze  describes  this  brain  very  thoroughly,  and  it  is  of  es- 
pecial interest  inasmuch  as  its  bearer  lived  to  the  age  of  five  years.  As  far 
as  any  history  may  be  depended  upon,  it  was  simply  this,  that  the  child 
appeared  normal  at  birth,  and  was  supposed  to  have  moved  its  hands ;  but 
this  statement  Schultze  questions,  and  states  that  later  on  both  arms 
were  in  a  condition  of  flexion  and  adduction.  The  legs  were  flexed,  and 
were  in  a  state  of  spastic  contracture  and  adduction.  The  child  never  had 
any  convulsions,  but  was  able  to  scream  and  to  suckle.  The  father  of  the 
child  noticed  that  there  was  no  sign  of  intelligence,  that  the  child  would  not 
take  hold  of  anything,  that  it  did  not  hear,  and  that  it  lay  constantly  with  its 
eyes  rolling  about,  and  with  its  head  retracted.  The  condition  was,  therefore, 
one  of  complete  idiocy,  with  paralysis  and  contractures  of  both  upper  and 
lower  extremities.     The  child  grew  weaker  and  weaker,  and  finally  died  of  a 


504 


THE  NERVOUS  DISEASES    OF   CHILDREN. 


bronchitis.     The  parents  of  this  child  had  six  others,  all  of  them  healthy. 
The  mother  of  the  child  was  addicted  to  drinking. 

Schultze  disputes  the  correctness  of  Kundrat's  views,  that  porencephaly 
is  the  result  of  anaemic  necrosis.  This  author  insisted  on  the  marked 
distinction  between  the  congenital  and  acquired  cases  of  porenceph- 
aly. If  the  porencephaly  is  of  intra-uterine  origin,  the  convolutions  are  so 
arranged  as  to  radiate  toward  the  margin,  or  toward  the  middle  of  the 
defect.  If  the  defect  is  an  irregular  one,  and  the  convolutions  are  not  ar- 
rayed in  any  definite  fashion  the  porencephaly  is  acquired  after  birth.     In 


A.  C.  C. 


r-  p.  c.  c. 


Fie 


128. — Brain  with  Large  Anterior  Defect.     A.  C.  C.  anterior  central  convolutions; 
P.  C.  C. ,  posterior  central  convolutions.     (Schultze.) 


this  question,  as  in  so  many  others,  there  is  some  truth  in  views  that  appear 
to  be  distinctly  divergent.  I  have  no  doubt  that  some  cases  of  porencephaly 
are  due  to  vascular  lesions,  and  others  to  inflammatory  processes  which  may 
possibly  start  from  the  meninges.  The  symmetrical  defect  in  Schultze's  case 
seems  to  me  more  likely  to  be  due  to  an  inflammatory  meningeal  process 
without  exudation,  at  a  relatively  early  stage  of  fcetal  development,  than  to  a 
destruction  secondary  to  vascular  disease.  In  Schultze's  case,  moreover,  the 
vascular  apparatus  was  found  to  be  entirely  normal.  The  question  arises, 
What  the  causes  of  an  intra-uterine  inflammatory  process  could  possibly  be  ? 
If  the  mother  had  passed  through  any  pyaemic  condition,  or  had  had  some 
form  of  purulent  disease  during  pregnancy  the  cause  of  such  inflammatory 
meningitis  or  encephalitis  would  not  be  far  to  seek ;  but  in  Schultze's,  as  in 


DEFECTIVE   DEVELOPMENT   OF   THE   BRAIN, 


505 


other  cases,  no  such  cause  could  be  made  out,  and  it  will  not  do  to  speculate 
upon  improbabilities.  The  readiest  explanation  is  to  assume,  as  many  of  the 
older  French  writers  assumed  with  regard  to  various  brain  defects,  that 
traumatism  plays  a  very  important  role,  and  that  traumatic  encephalitis  dur- 
ing the  intra-uterine  period  may  be  the  actual  cause  of  some  of  these  poren- 
cephalic conditions.  Schultze  mentions  that  spasmodic  contractions  of  the 
uterus  without  any  extraneous  disturbance  may  be  a  possible  cause  of  such 
traumatic  changes  in  the  foetal  brain.  In  his  own  case  Schultze  is  inclined  to 
consider  the  possibility  of  a  degeneration  or  obliteration  of  some  of  the 


Fig.  129 Same  Brain  as  in  Preceding  Figure,  Viewed  from  the  Base.     (Schultze.) 


blood-vessels,  or  even  of  an  encephalitis,  as  a  direct  result  of  alcoholic  ten- 
dencies of  the  mother.  In  the  most  recently  reported  cases  of  porencephaly 
of  the  parietal  region  there  seems  to  be  atrophy  of  all  motor  fibres  from  the 
cortex  to  the  medulla  oblongata.     (In  eleven  of  fourteen  cases. — Anton.) 

Acquired  Porencephaly  is  a  condition  due  to  early  meningeal 
hemorrhage.  This  in  turn  is  the  result  of  compression  of  the  skull  during 
labor.  The  symptoms  associated  with  these  brain  conditions  will  be  found 
described  under  the  heading  of  Cerebral  Birth  Palsies.  The  meningeal  hem- 
orrhage is  in  many  cases  most  severe  under  the  parietal  eminences,  and  over 
the  motor  areas.  For  this  reason  double  spastic  cerebral  palsy  is  a  natural 
result  of  a  defect  which  develops  in  the  region  occupied  by  the  motor  cen- 
tres. This  region  is  also  supplied  by  the  middle  cerebral  artery,  which  is 
most  liable  to  all  vascular  disease.  The  porencephalic  defect  due  to  men- 
38 


506 


THE   NERVOUS   DISEASES    OF   CHILDREN. 


ingeal  hemorrhage  may  be  present  in  both  hemispheres,  or  it  may  exist  in 
only  one-half  of  the  brain.  The  meningeal  hemorrhage,  if  sufficiently  se- 
vere, leads  to  a  compression  of  the  cortical  substance  ;  atrophy  of  the  cortex 
follows,  upon  this  the  substance  shrinks  considerably,  and  the  cerebro- 
spinal fluid  is  increased  either  as  a  result  of  inflammatory  action,  or  of  an 
effort  at  compensation.  The  pia  and  the  cortex  are  apt  to  become  aggluti- 
nated, and  thus  local  cystic 
conditions  with  extreme  loss 
of  substance  may  readily  oc- 
cur, the  cyst  occupying  the 
place  which  should  be  filled 
by  normal  brain  substance. 

Through  the  kindness  of 
Dr.  Peterson  the  author  is 
enabled  to  insert  an  illustra- 
tion (Fig.  130)  of  the  brain  of 
a  child  that  died  at  the  age 
of  twenty  months.  The  child 
had  been  subject  to  convul- 
sions and  had  presented  com- 
plete spastic  paralysis  of  all 
four  extremities ;  the  head 
measurements  were  extreme- 
ly small  (circ.  32.5  ctm.).  Over 
each  hemisphere  a  large  por- 
tion of  the  convolutions  was 
wanting.  The  vacuum  was 
filled  partly  by  subdural  fluid, 
and  partly  by  the  bulging  of 
each  ventricle,  for  there  was  also  internal  hydrocephalus.  Peterson  thinks  the 
condition  due  to  a  double  meningeal  hemorrhage,  but  the  defect  corresponds 
so  nearly  to  the  distribution  of  the  middle  cerebral  arteries  that  obliteration 
of  these  blood-vessels  early  in  fcetal  life  may  have  been  the  prime  cause. 


Fig.  130. — Large  Double  Porencephalic  Defect. 
Child  lived  to  age  of  twenty  months.  (From  a 
brain  kindly  furnished  by  Dr.  Peterson.) 


MICROCEPHALUS. 


In  addition  to  the  anomalies  of  brain  structure  in  which 
one  or  more  parts  may  be  defective,  we  have  to  consider 
that  condition  in  which  the  brain  as  a  whole  shows  a  de- 
fective development,  failing  to  attain  to  the  normal  size. 
There  is,  as  a  rule,  a  correspondingly  small  development  of 
the  skull,  and  to  this  small  head  the  term  "  microcephalus  " 
is  given.  While  a  microcephalus  is  bound  to  harbor  an  ab- 
normally small  brain,  we  must  not  forget  that  a  skull  of 


DEFECTIVE   DEVELOPMENT   01    THE   BRAIN.  S°7 

normal  size  may  also  contain  a  small  brain,  the  cranial 
cavity  under  such  conditions  being  filled  up  by  an  excess 
of  fluid.  Microcephalus  is  evidently  the  result  of  a  num- 
ber of  different  processes.  It  has  been  a  habit  to  explain  it 
altogether  on  Virchow's  theory,  that  the  smallness  of  the 
skull  was  due  to  a  premature  ossification  of  the  cranial 
sutures,  which  interfered  with  the  proper  expansion  of  the 
skull,  and  that  in  consequence  of  this  stunted  growth  of 
the  skull  the  growth  of  the  brain  was  arrested.  This  ex- 
planation will  hold  good  for  a  considerable  number  of  cases, 
but  I  am  firmly  convinced  not  for  all.  We  must  allow  also 
for  those  cases  of  microcephalus  due  to  defective  cerebral  de- 
velopment during  the  intra-uterine  or  during  the  early  extra- 
uterine period.  For  reasons  which  we  cannot  always  fathom, 
the  brain  does  not  grow  and  the  skull  remains  small.  The 
children  are  small-skulled  and  small-brained.  In  another 
series  of  cases  the  microcephalus  is,  I  am  convinced,  the 
result  of  early  vascular  lesions.  I  have  in  mind  several 
cases  of  children  who  were  born  normally,  whose  cerebral 
capacity  was  on  a  par  with  that  of  other  children  up  to 
the  age  of  six,  seven,  or  nine  months.  Then  convulsions 
set  in,  probably  resulting  in  extensive  meningeal  hemor- 
rhage, or,  let  us  say,  in  an  extensive  pachymeningitis  hem- 
orrhagica. In  consequence  of  this  the  cortex  was  com- 
pressed ;  it  became  atrophic  and  sclerotic  and,  remaining 
small,  the  skull  did  not  expand  in  normal  fashion.  Such 
children  become  microcephalic  after  the  first  year  of  life, 
or  later. 

This  leads  us  to  a  question  which  has  been  much  discussed  and  unsatis- 
factorily answered,  whether  the  growth  of  the  skull  depends  upon  the  growth 
of  the  brain  or  vice  versa  (Meynert,  Virchow,  Lucas).  The  two  hold  a 
very  close  relation  to  one  another.  If  premature  stenosis  sets  in  it  is  un- 
questionably sufficient  to  account  for  a  small  brain  ;  but,  on  the  other  hand, 
a  growing  brain  evidently  helps  to  expand  a  normal  skull.  The  fact  that 
defective  brains  are  often  covered  by  normal  skulls  if  the  defect  has  been 
compensated  by  a  local  accumulation  of  fluid,  would  seem  to  prove  that  as 
long  as  the  same  mechanical  conditions  are  present  the  growth  of  the  skull 
remains  tolerably  normal  (Gudden). 

In  order  that  we  may  be  able  to  determine  when  a  skull 
may  be  termed  microcephalic,  we  must  refer  to  the  meas- 


508  THE  NERVOUS  DISEASES   OF  CHILDREN. 

urements  of  normal  heads  at  certain  ages  (see  Introductory 
Chapter).  If  a  head  which  is  supposed  to  be  microcephalic 
fall  far  short  of  such  average  measurements  the  diagnosis 
of  microcephalus  may  be  safely  made  ;  but  the  head  may 
be  developed  in  proportion  to  the  rest  of  the  body  ;  a  small 
measurement  in  a  child  need  not  necessarily  imply  a  con- 
dition of  microcephalus,  unless  the  measurement  of  the 
skull  is  below,  while  other  measurements  of  the  body  are 
fully  up  to,  the  average  in  other  children. 

The  general  measurements  of  the  skull  are  not  always  sufficient  to  prove 
whether  the  brain  is  small  or  not.  Each  part  of  the  skull  should  be  inspected 
per  se.  Thus  I  have  seen  extremely  small  and  receding  foreheads  with  un- 
usually large  occiputs.  This  would  tend  to  give  a  measurement  of  a  general 
circumference  quite  up  to  the  standard,  yet  on  closer  inspection  there  would 
be  no  doubt  that  the  frontal  portions  of  the  skull  and  of  the  brain  were  poorly 
developed,  whereas  the  occipital  portions  were  either  normally  developed  or 
caused  to  bulge  by  the  accumulation  of  hydrocephalic  fluid  within  the  ven- 
tricles. 

In  several  other  heads  which  the  author  has  examined  this  defect  in  the 
anterior  portion  was  extremely  marked,  and  as  the  frontal  portion  of  the 
brain  has  least  to  do  with  motor  and  sensory  functions,  and  more  with  the 
general  intellectual  development  of  the  child,  we  can  see  the  important  bear- 
ing such  facts  have  upon  the  prognosis  of  a  child's  future  mental  develop- 
ment. A  frontal  microcephalus  may  be  present  even  though  the  cranial 
measurements  be  up  to  the  average. 

The  symptoms  due  to  microcephalus  are  described 
under  the  heading  of  idiocy,  and  in  the  chapter  on  the  Cere- 
bral Palsies  of  Children.*  In  this  connection  we  may  refer, 
however,  to  efforts  made  within  recent  years  to  correct 
these  cranial  deformities  on  the  supposition  that  if  the  size  of 
the  cavity  could  be  enlarged  the  brain  would  have  a  more 
favorable  opportunity  for  further  growth.  Such  reasoning 
could  apply  merely  to  those  skulls  in  which  the  chief 
trouble  lies  in  the  premature  synostosis  of  the  sutures.  If 
there  is  an  inherent  defect  in  the  development  of  the  brain, 
or  if  the  brain  growth  has  been  checked  as  the  result  of  ac- 
quired disease,  such  treatment  is  not  applicable.  The  great 
difficulty,  however,  is,  that  it  is  not  always  easy  to  deter- 

*  Of  course  microcephalic  heads  are  at  times  found  in  persons  of  tolerable  cerebral 
development. 


DEFECTIVE  DEVELOPMENT  OE  THE  BRAIN.  509 

mine  to  which  one  of  these  causes  the  defective  cerebral 
development  is  due.  Lannelongue  suggested  many  years 
ago  that  in  the  case  of  microcephalic  skulls  a  longitudinal 
opening  be  made  in  one  or  both  halves  of  the  skull,  thus 
practically  increasing  the  size  of  the  intra-cranial  cavity,  or 
at  least  diminishing  the  pressure  within  this  cavity.  My 
experience  has  not  been  at  all  favorable  to  Lannelongue's 
doctrine,  and  I  am  willing  to  say  that  there  is  not  the  least 
reason  to  advise  or  encourage  the  operation,  at  least  accord- 
ing to  the  method  proposed  by  Lannelongue.  This  French 
surgeon  operated  upon  a  number  of  skulls,  and  claimed  that 
after  operation  the  children  showed  some  signs  of  increased 
intelligence  and  were  greatly  benefited  by  the  operation. 
Other  surgeons  have  had  but  few  favorable  results  to  show, 
and  of  the  cases  operated  upon  there  were  not  many  in 
which  a  small  brain  was  due  to  smallness  of  skull.  I  have 
had  three  children  operated  upon,  and  on  one  patient  two 
operations  were  performed.  Two  patients  with  micro- 
cephalic skulls  due  to  premature  ossification  of  the  sutures, 
died  very  shortly  after  the  operation.  The  third  case 
was  a  child,  two  years  of  age,  whose  skull  had  evidently 
undergone  a  premature  synostosis.  It  survived  the  opera- 
tion and  showed  a  little  improvement,  inasmuch  as  it  began 
to  play  with  other  children  in  the  hospital  ward,  but  dur- 
ing the  four  months  after  operation  did  not  learn  either 
to  talk,  stand,  or  to  walk.  The  improvement  was,  in  fact, 
very  slight  indeed.  Wishing  to  give  the  method  a  further 
test,  the  child  was  operated  upon  a  second  time,  and  an 
opening  similar  to  the  first  was  made  in  the  opposite  half  of 
the  skull.  The  child  died  from  "shock"  four  hours  after 
the  operation  was  finished. 

Figure  131  is  inserted  to  show  tne  condition  of  the  skull* 
after  the  second  operation.  It  will  be  seen  at  first  glance 
that  the  original  opening  that  was  made  became  very  much 
smaller  after  four  months ;  that  a  tense  fibrous  tissue  was 
formed  over  this  opening,  and  that  therefore  four  months 
after  the  initial  operation  the  brain  was  not  under  condi- 
tions much  more  favorable  than  those  which  preceded  the 

*This  skull  was  demonstrated  by  Dr.  A.  Jacobi,  at  the  International  Congress,  held 
in  Rome,  1894.     In  the  last  ten  years  the  author  has  not  advised  this  operation. 


5io 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


first  operation.  I  would  therefore  conclude  from  my  own 
experience  *  that  Lannelongue's  operation  is  not  likely  per- 
manently to  increase  the  intra-cranial  capacity,  and  further- 
more, that  operations  upon  young-  children  are  extremely 


Fig.  131. — Skull  of  a  Child  Two  Years  of  Age,  showing  (on  the  left  of  the  figure)  the 
Narrowing  of  the  Opening  Made  According  to  Lannelongue's  Method  Four  Months 
Previous  to  Death.     Death  ensued  four  hours  after  second  operation. 

dangerous,  the  children  often  dying  from  shock,  or  from 
the  loss  of  blood. 

If  we  wish  to  utilize  Lannelongue's  idea  it  will  be  better 
to  make  a  circular  opening  over  that  part  of  the  brain 
which  from  the  symptoms  in  the  case  would  appear  to  be 
deficient.  In  children  whose  frontal  lobes  are  stunted  in 
growth,  circular  openings  in  the  frontal  or  in  the  parietal 
bones  would  give  a  very  large  measure  of  relief,  and  I  am 

*  Bourneville,  Jacobi  and  almost  every  one  else  have  come  to  similar  conclusions. 


DEFECTIVE   DEVELOPMENT  OF   THE   BRAIN".  511 

confident  that  if  the  operation  is  entirely  aseptic  cerebral 
hernia  need  not  be  feared.  The  bone  should  not  be  re- 
placed, but  it  will  be  well  to  provide  such  children  with 
some  form  of  external  protection,  or  else  the  danger  of  di- 
rect injury  to  the  brain  from  falls  would  be  very  great 
indeed.  The  author  wishes  once  more  to  emphasize  the 
fact  that  there  are  relatively  few  children  in  whom  a  small 
skull  is  the  primary,  or  even  the  most  important,  cause  of 
arrested  development  of  the  brain.  The  fault  lies  chiefly 
with  the  brain  itself  and  cannot  be  remedied  by  operation. 

Partial  Cerebral  Defects. — In  speaking  of  the  con- 
dition of  the  brain  in  microcephalic  children  I  have  alluded 
to  the  fact  that  a  part  of  the  brain,  and  not  necessarily  the 
entire  brain,  may  be  checked  in  its  development.  This  is 
particularly  marked  with  regard  to  the  frontal  and  the 
occipital  lobes.  These  conditions  deserve  special  atten- 
tion, moreover,  because  some  of  them  are  attended  by 
symptoms  which  enable  us  to  diagnosticate  during  life  the 
exact  area  of  defective  development.  It  is  fair  to  assume 
that  if  the  frontal  lobes  are  wanting,  intelligence  will  suffer 
most,  whereas  the  movements  of  the  child  will  be  normal, 
sensation  will  be  undisturbed,  and  all  the  special  senses,  ex- 
cept possibly  the  olfactory  sense,  will  be  well  developed.  It 
is  not  claimed  that  intelligence  is  altogether  the  property  of 
the  frontal  lobes,  for  the  various  functions  that  are  so  essen- 
tial to  normal  mental  faculties  are  stored  in  the  most  diverse 
portions  of  the  brain,  and  a  defect  in  one  or  more  of  these 
sensory  divisions  of  the  brain  in  young  children  will  natu- 
rally interfere  with  normal  mental  development.  If  the  de- 
fect is  in  the  motor  areas,  the  disturbances  of  motion  and  of 
sensation  will  naturally  point  out  the  defective  area.  While 
porencephaly  is  extremely  frequent  in  this  region,  I  have 
not  yet  seen  a  single  brain  that  exhibited  normal  develop- 
ment of  its  fissures  and  convolutions  in  all  but  the  motor 
region. 

Defective  Development  of  the  Occipital  Lobe. — ■ 
Defective  development  of  the  occipital  lobe,  as  indicated  by 
an  atrophy  of  the  gyri  of  this  lobe,  is  well  illustrated  by  the 
case  represented  in  Fig.  132,  which  has  been  reported  by 
Peterson.     "  The  patient  was  a  female,  twenty-eight  years 


512 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


of  age,  and  was  from  birth  an  idiot,  with  epilepsy  and  left 
hemiplegia  (slight)  ;  she  was  almost  blind,  being  able  only 
to  distinguish  light  from  darkness.  Both  occipital  lobes 
were  greatly  atrophied ;  the  loss  of  tissue  was  made  up  for 
by  the  widely  dilated  posterior  horns  of  the  ventricles. 
The  cerebellum  projected  far  beyond  the  posterior  lobes ; 


Fig.  132.* — Brain  of  a  Woman  Aged  Twenty-eight  years,  Idiot  from  Birth  and  almost 
Blind.  Defective  development  of  both  occipital  lobes,  which  appear  to  be  mere  ap- 
pendages to  the  more  normal  anterior  portions.  Drawing  made  from  specimen  ;  pia 
of  left  half  not  removed. 


the  atrophy  and  sclerosis  extended   partly  into  the   motor 
area  of  the  right  side." 

Defective  development  of  the  larger  ganglia  is  not  met 
with  in  cases  in  which  there  are  no  other  large  cerebral  de- 
fects.    This  statement  must  be  accepted  with  some  reserve, 

*  The  author  is  indebted  to  Dr.  Peterson  for  permission  to  have  the  specimen  copied. 


DEFECTIVE  DEVELOPMENT  OF   THE    BRAIN.  513 

for  little  attention  has  been  paid  to  this  subject,  and  of  the 
defective  brains  that  have  been  examined  the  records  say 
very  little  about  the  ganglionic  region. 

The  author  has  had  occasion  to  diagnosticate  defective 
development  of  the  cerebellum.  The  diagnosis  was  made 
in  a  girl,  eight  years  of  age,  born  without  difficulty  and  at 
full  term.  The  child  had  never  learned  to  walk  or  to  talk. 
There  was  not  a  sign  of  spasticity  or  of  actual  paralysis.  If 
the  girl  was  placed  on  her  feet  she  would  fall  over  at  once 
to  the  right.  Equilibrium  could  not  be  maintained  while 
the  child  was  in  the  erect  position.  Sitting  up  in  bed  or  on 
a  table  she  was  able  to  move  the  various  parts  of  the  body. 
The  knee-jerks  were  absent ;  hearing  and  sight  normal. 
There  was  a  condition  of  complete  idiocy.  The  defect  of 
the  cerebellum  was  evidently  part  of  a  general  brain  defect. 
The  case  bore  a  superficial  resemblance  to  Friedreich's  dis- 
ease, but  could  be  differentiated  from  it  by  the  early  onset 
and  the  development  of  idiocy  very  soon  after  birth. 

Agenesis  Corticalis. — In  the  conditions  previously 
described  gross  defects  of  the  entire  brain,  or  of  parts  of 
the  brain,  have  been  referred  to.  In  the  condition  of  agene- 
sis corticalis  the  size  and  weight  of  the  brain  may  be  en- 
tirely normal.  The  changes  to  be  observed  are  those  dis- 
covered on  microscopical  examination.  The  only  outward 
indication  of  the  imperfect  development  in  the  external 
configuration  of  the  brain  is  abnormal  fissuration.  The 
fissure  of  Rolando  dips  straight  into  the  fissure  of  Sylvius, 
without  leaving  a  small  convolution  between  the  two  fis- 
sures as  in  normal  brains.  (See  Fig.  133.)  The  interparietal 
fissure  extends  farther  back  than  normal  into  the  occipital 
lobe;  the  island  of  Reil  may  be  entirely  exposed  instead  of 
being  obscured  from  view  by  the  margins  of  the  fissure  of 
Sylvius.  Such  differences  as  these  are  characteristic  of  a 
lower  order  of  brain  development  (criminal  brains,  for  in- 
stance). On  minute  examination  of  such  brains  that  have 
been  hardened,  the  large  nerve-cells  in  the  cortex  are  seen 
to  be  distorted,  their  contours  not  as  well  marked  as  in  the 
normal  brain,  the  protoplasmic  contents  are  found  in  va- 
rious stages  of  disintegration.  The  nuclei  are  either  sub- 
divided   or   destroyed,   and   the   fine   nerve -processes,    so 


5H 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


beautifully  distinct  in  the  normal  cells,  have  disappeared 
entirely.  Sections  through  the  cortical  tissue  of  any  part 
of  the  hemispheres  reveal  practically  the  same  condition. 
The  anatomical  study  of  two  cases,  made  some  years  ago, 
led  to  the  recognition  of  a  special  type  of  idiocy  with  blind- 
ness and  ending  in  marasmus.  (See  section  on  Amaurotic 
Family  Idiocy.)  It  has  been  definitely  ascertained  that  the 
agenesis  corticalis  is  part  of  a  degeneration  of  the  gray  mat- 
ter of  the  entire  central  nervous  system. 


y  t-2 


Fig.  133 — Photograph  of  Brain  of  Author's  First  Case  of  Amaurotic  Family  Idiocy. 
The  histological  condition  was  described  as  an  "  agenesis  corticalis."  In  the  above 
figure,  the  confluence  of  fissures  and  the  exposure  of  the  Island  of  Reil  are  the  signs 
of  a  low  order  of  cerebral  development.  Through  the  hardening  process  the  condi- 
tions have  been  accentuated  a  little,  but  they  were  present  in  the  fresh  specimen. 
At  x,  and  in  the  frontal  lobes,  sections  had  been  removed  for  histological  examina- 
tion.    Other  letters  refer  to  fissures. 


MACROCEPHALUS. 

This  term  may  be  applied  equally  well  to  an  increase  in  the  size  of  the 
head  due  to  hydrocephalus,  as  to  those  rarer  conditions  in  which  an  in- 
crease in  the  volume  of  the  brain  has  brought  about  a  corresponding  in- 
crease in  that  of  the  skull.  Hypertrophy  of  the  brain  is  very  rare  indeed. 
While  some  maintain  that  the  increased  size  of  the  brain  is  due  to  a  prolif- 
eration of  the  neuroglia,  others  believe  that  it  is  the  result  of  an  increase 
in  all  the  elementary  structures  of  the  brain,  but  the  histological  examination 
has  been  made  in  so  few  cases  that  the  point  has  not  been  decided.     Steffen 


DEFECTIVE  DEVELOPMENT   OF   THE   BRAIN.  515 

remarks  correctly  enough  that  if  the  increased  size  were  due  to  a  prolifera- 
tion of  the  neuroglia,  this  proliferation  would  come  to  a  standstill  and  a  par- 
tial atrophy  and  sclerosis  would  be  the  natural  result,  but  in  those  brains 
which  have  been  examined  post  mortem  true  hypertrophy  was  found  and  no 
indications  of  a  sclerotic  condition.  Rilliet  and  Barthez,  Gerhardt  and  others, 
evidently  confound  cases  of  interstitial  encephalitis  with  hypertrophy  of  the 
brain.  Steffen  is  correct  in  insisting  that  the  term  should  be  restricted  to 
those  in  which  there  is  a  true  hypertrophy  of  a  part  of,  or  of  the  whole  brain. 
In  consequence  of  the  increased  growth  of  the  brain  the  resisting  skull 
causes  increased  pressure  ;  as  a  result  of  this  the  blood-vessels  are  narrowed 
and  the  circulation  is  impaired,  whence  it  follows  that  the  brain  is  pale  and 
anaemic  ;  the  convolutions  are  often  considerably  flattened,  and  the  fissures 
become  more  or  less  indistinct.  The  ventricles  may  be  greatly  compressed, 
or  almost  obliterated.  By  compression  from  without  the  cerebro-spinal  fluid 
is  driven  largely  out  of  the  ventricles.  As  long  as  the  fontanelles  remain  open 
the  danger  from  compression  is  not  as  great  as  it  is  in  later  years  when  the 
entire  skull  represents  an  unyielding  mass.  As  long  as  the  fontanelles  re- 
main open  a  true  hypertrophy  can  be  differentiated  from  a  chronic  hydro- 
cephalus. The  hydrocephalus  increases  more  rapidly  as  a  rule  than  the 
hypertrophy  does.  The  fontanelle  will  be  more  distinctly  pulsating  in  hydro- 
cephalus than  in  the  other  condition.  The  form  of  the  head  will  vary  much 
in  these  two  processes.  In  the  case  of  an  hypertrophied  brain  the  entire 
skull  will  be  equally  enlarged  ;  whereas  in  hydrocephalus  the  accumulation 
of  fluid  in  the  anterior  or  the  posterior  horns  of  the  ventricles  tends  to  a  special 
increase  in  the  frontal  and  in  the  occipital  portion  of  the  skull.  The  sutures 
may  be  forced  apart  by  the  increase  in  fluid. 

The  clinical  symptoms  of  this  condition  are  not  very  different  from  those 
found  in  chronic  hydrocephalus  ;  a  considerable  mental  development,  or  even 
normal  mental  development,  is  compatible  with  hypertrophy  of  the  brain 
tissue,  and  if  the  fontanelles  are  not  closed  the  increase  in  the  size  of  the 
brain  does  not  entail  a  very  considerable  increase  of  pressure,  but  it  is  false 
to  suppose  that  children  with  hypertrophied  brains  would  naturally  be 
brighter  than  those  whose  brains  are  of  normal  dimensions.  The  hyper- 
trophy is  self-limited  by  reason  of  the  additional  pressure  it  causes. 

If  the  size  of  the  head  increases  very  much,  children  are  unable  to  carry 
the  head,  feel  the  need  of  supporting  it,  become  apathetic,  morose,  and  have 
a  tendency  to  fall  ;  if  the  anaemia  increases,  convulsions,  disturbances  of  vi- 
sion, vomiting,  and  a  gradual  cessation  of  all  the  faculties  may  precede  death, 
which  occurs  after  an  indefinite  period  of  time,  unless  brought  about  more 
rapidly  by  an  intercurrent  disease.  It  will  not  do  to  devote  too  much  atten- 
tion to  this  condition,  which  is  of  extremely  little  practical  value.  The  diag- 
nosis of  hypertrophied  brain  will  be  made  much  more  frequently  at  the  post- 
mortem table  than  in  the  wards  of  a  hospital  or  in  private  practice. 

Defective  Development  of  the  Cranial  Nerve 
Nuclei  should  be  considered  in  conjunction  with  the  larger 


516  THE  NERVOUS  DISEASES   OF   CHILDREN. 

defects  described  above.  These  cases  have  received  but 
very  little  attention,  yet  they  are  of  great  interest  both 
from  a  clinical  and  from  an  anatomical  stand-point.  The 
nuclei  most  frequently  affected  are  those  connected  with  the 
nerves  governing  the  ocular  muscles.  Cases  of  congenital 
ptosis,  unilateral  or  bilateral,  are  so  common  that  every 
one  can  recall  persons  so  affected.  Forms  of  congenital 
deficiencies  in  facial  innervation  come  under  this  heading. 
Gowers  has  made  a  short  note  of  them  under  the  heading 
of  "  Infantile  Oculo-facial  Palsy."  Moebius  collected  over 
forty  cases  of  this  class  and  reported  upon  them  in  the  year 
1892  ;  while  Schapringer  gave  a  most  intelligent  descrip- 
tion of  a  similar  condition  which  he  termed  "  Congenital 
Bilateral  Pleuroplegia  (Paralysis  of  Lateral  Movements) 
and  Facial  Palsy."  His  case,  which  we  shall  give  some- 
what more  in  detail,  shows,  however,  that  his  title  did  not 
exhaust  the  clinical  symptoms  of  the  case,  for  there  was  evi- 
dence of  involvement  of  the  fifth  nerve  as  well  as  of  the  sixth 
and  seventh.  I  should,  therefore,  prefer  to  give  to  all  these 
diseases  the  proper  designation  of  "  Congenital  Nuclear 
Palsy."  That  there  is  in  these  conditions  an  actual  defect 
in  the  development  of  the  cranial  nuclei  there  can  be  little 
doubt,  and  that  these  defects  are  associated  with  other  con- 
ditions of  defective  development  is  proved  by  the  occur- 
rence of  these  congenital  nuclear  palsies  with  imbecility  or 
idiocy.  (Oppenheim,  Gowers,  Heubner,  and  the  author.) 
I  have  seen  several  interesting  cases  of  congenital  nuclear 
palsy  in  patients  afflicted  with  scleroderma  and  myxcedema. 
We  shall  be  justified,  therefore,  in  relegating  to  this  class 
of  cases  all  those  patients  in  whom  there  is  a  congenital 
defect  of  a  unilateral  or  bilateral  character  implying  insuffi- 
cient innervation  of  the  muscles  governed  by  any  of  the 
cranial  nerves.  The  forms  that  are  most  frequently  observed 
are  congenital  ptosis,  ophthalmoplegia  externa,  partial  or 
complete,  facial  paralysis,  and  paralysis  of  the  tongue.  A 
close  clinical  and  anatomical  relationship  would  seem  to 
me  to  exist  between  these  cases  of  congenital  nuclear  palsy, 
and  cases  of  hereditary  progressive  muscular  atrophy,  pos- 
sibly also  between  these  nuclear  palsies  and  those  with  local 
muscular  defects. 


DEFECTIVE   DEVELOPMENT   OF   THE   BRAIN. 


517 


The  symptoms  of  these  types  will  naturally  vary  very  much.  I  propose 
giving  a  short  summary  of  Schapringer's  case,  which  exhibits  a  more  con 
association  of  symptoms  than  any  other  with  which  I  have  become  acquainted. 
The  history  in  brief  is  this  :  A  girl,  eight  years  of  age  (Fig.  134),  who  was 
suffering  at  the  time  from  a  slight  bronchial  trouble,  was  examined  by  Dr. 
Schapringer  in  1889.  The  child  was  of  normal  development,  and  of  average 
intelligence,  although  she  was  not  sent  to  school,  and  had  not  yet  learned 
to  read.  In  walking  she  stumbled  and  fell  more  frequently  than  other  chil- 
dren of  the  same  age.  She  states  that  this  is  in  consequence  of  a  weakness 
in  the  legs,  and  not  on  account  of  the  visual  disturbance.  Her  face  is  pale, 
expressionless,  and  mask-like.  The  naso-labial  fold  is  absent  on  both  sides, 
there  is  not  the  slightest  indication  of 
folds  in  the  integument  of  the  face 
either  in  laughing  or  crying.  Her 
laughing  and  crying  sounds  cannot 
easily  be  distinguished.  She  cannot 
close  her  lips.  Substitutes  Unguals  for 
labials,  "  tata  "  for  "  papa,"  etc.  The 
right  angle  of  the  mouth  is  drawn 
downward  and  outward.  Whistling, 
blowing,  etc.,  are  impossible.  She  is 
not  able  to  wrinkle  the  forehead  either 
vertically  or  transversely.  When  her 
eyes  are  open  the  ordinary  amount  of 
eyeball  is  uncovered.  Quite  often  one 
lid  hangs  a  little  lower  than  its  fellow, 
but  the  eyes  can  be  fully  and  com- 
pletely opened,  proving  that  both  leva- 
tores  palpebrarum  are  not  involved. 
Complete  closure  of  the  eyes  is  impos- 
sible, although  the  lids  can  upon  forci- 
ble effort  be  closely  approximated  to 

one  another.     There  is  a  distinct  epicanthus,  the  caruncle  and  plica  semilu- 
naris are  only  slightly  developed  on  both  sides. 

Ordinarily  the  axes  of  the  eyes  are  parallel,  frequently,  however,  the  right 
eye  is  directed  a  little  more  upward  and  slightly  outward.  She  is  able  to 
follow  an  object  with  both  eyes  if  it  be  lowered  or  elevated  in  the  median 
line.  If  the  object  be  approached  to  her  face,  keeping  it  in  the  saggital  plane 
she  follows  it  readily,  and  she  will  keep  her  eyes  fixed  upon  a  finger  held 
near  the  nose  for  a  long  period  without  fatigue.  These,  however,  are  all  the 
movements  of  which  the  eyeball  is  capable.  The  two  internal  recti  contract 
freely  if  required  in  order  to  bring  the  eyes  into  convergence  for  near  objects. 
If,  however,  one  of  these  muscles  be  required  to  act  in  unison  with  the  ex- 
ternal rectus  of  the  opposite  eye  for  a  conjugate  lateral  vision,  there  will  be 
found  an  utter  inability  to  do  so.  The  two  external  recti  muscles  are  either 
completely  paralyzed  or  very  deficient.  The  internal  recti  are  not  wholly 
paralyzed.     When  the  child  wishes  to  see  an  object  situated  to  the  side 


Fig.  134. — Girl,  Eight  Years  Old,  with 
Congenital  Pleuroplegia,  ( Scha- 
pringer.) 


5 18  THE  NERVOUS  DISEASES   OF   CHILDREN. 

of  the  median  plane,  instead  of  rotating  the  eyes  she  turns  her  entire  head. 
The  fundus  appears  normal,  except  that  the  vessels  are  a  little  more 
tortuous  than  usual.  The  functions  of  the  ciliary  muscles  are  normal. 
There  is  no  strabismus  convergens.  The  size  and  mobility  of  the  pupils 
are  normal.  When  the  tongue  is  protruded  it  appears  to  the  left  of  the  me- 
dian line ;  the  left  half  is  a  trifle  smaller  than  the  right.  When  eating,  the 
child  is  obliged  to  use  its  finger  to  dislodge  food  from  the  cheeks.  She  is 
unable  to  masticate  hard  substances,  such  as  crusts.  She  can  move  the 
lower  jaw  laterally  toward  the  right  side,  but  not  toward  the  left ;  thus  indi- 
cating a  paralysis  of  the  right  pterygoid  muscle.  She  has  uvula  bifida.  There 
is  an  hypertrophied  condition  of  the  canine  ligament  of  the  right  side,  and 
normal  development  of  the  left.  In  addition  to  these  congenital  defects  the 
author  mentions  a  deflection  of  the  distal  phalanges  of  the  index-fingers  at  an 
angle  of  about  one  hundred  and  fifty  degrees  in  the  direction  of  the  middle 
finger.  He  interprets  this  also  to  be  a  congenital  abnormality.  The  child  has 
the  condition  known  as  "  funnel  chest." 

Reviewing-  the  symptoms,  which  I  have  quoted  very 
freely  from  Dr.  Schapringer's  paper,  it  is  evident  that  the 
congenital  defects  involve  the  motor  branch  of  the  fifth,  the 
hypoglossal,  and  the  facial  nerve,  also  the  tracts  which  gov- 
ern the  conjugate  lateral  movements,  viz.,  the  third  and 
sixth  nerve  nuclei,  and  the  nerve-tracts  connecting  the 
same.  The  only  criticism  to  be  passed  upon  this  interest- 
ing case  of  Dr.  Schapringer's  is  that  while  he  has  invented 
a  good  term  for  the  paralysis  of  the  lateral  movements, 
the  case  presents  other  symptoms;  and  that  the  congeni- 
tal bilateral  pleuroplegia  is  the  most  prominent  symptom 
of  the  condition  which  we  might  define  in  an  impartial 
way  as  one  of  bilateral  congenital  nuclear  palsy.  Cases  of 
this  description  are  evidently  rare,  for  Schapringer  had  been 
able  to  collect  but  four  others  like  the  one  he  described. 

During  the  past  ten  years  a  number  of  authors  have 
reported  instances  of  congenital  unilateral  facial  palsy,  pre- 
sumably due  to  defective  development  of  the  nucleus. 
Schultze  described  this  condition  in  a  girl  of  five  years, 
Bernhardt  in  a  man  of  twenty-four  years ;  and  Remak  has 
given  an  account  of  a  young  man  of  eighteen  years,  who 
presented  a  congenital  defect  of  the  platysma  myoides, 
associated  with  slight  bilateral  ptosis,  and  a  limitation  of 
ocular  movements  upward.  Surgical  treatment  only  is 
worth  considering. 


DISEASES   OF   THE   MIND. 

CHAPTER  XXIX. 

INSANITY. 

In  the  preceding  chapters  we  have  discussed  the  or- 
ganic  diseases  of  the  brain,  and  have  touched  upon  con- 
ditions due  to  arrest  of  development  and  to  morbid  affec- 
tions of  the  central  nervous  system  during  the  period  of  early 
growth.  Mental  disorders  may  be  similarly  subdivided. 
Idiocy  represents  the  psychic  derangement  due  to  arrested 
development ;  it  is  so  distinct  and  so  large  a  subject  that  it 
will  be  treated  separately.  Insanity  includes  the  morbid 
conditions  coming  on  after  a  fair  degree  of  mental  develop- 
ment has  been  reached,  and  constitutes  the  subject-matter 
of  this  chapter. 

Insanity  in  the  child  resembles  closely  that  in  the  adult.  Such  differ- 
ences as  do  exist  are  dependent  upon  the  unusual  sensitiveness  and  irritabil- 
ity of  the  youthful  mind,  and  upon  the  paucity  of  concepts  which  have  been 
formed  prior  to  the  development  of  the  insanity.  At  a  very  early  day  sensor}' 
impressions  become  fixed ;  hallucinations  are  possible,  therefore,  in  very  young 
children.  But  the  normal  mind  does  not  develop  systematized  concepts  nor 
the  logical  faculty  during  the  first  few  years  of  life,  and  prior  to  this  stage  of 
mental  development  delusions— faulty,  unfounded  beliefs— cannot  exist.  The 
delusions  of  an  insane  child  will  differ  from  those  of  the  adult  in  view  of  the 
limited  ideas  it  possessed  during  the  period  of  mental  health.  Its  delusions 
will  be  concerned  largely  with  its  own  individuality  and  its  relation  to  the 
family,  its  teacher,  and  its  God  ;  but  the  delusions  of  the  adult  in  regard  to 
his  wealth,  his  position  in  the  religious,  social,  and  political  world,  are  entirely 
foreign  to  the  child's  mind. 

The  normal  child  lacks,  moreover,  the  judgment  and  the  power  of  self- 
restraint  by  which  the  adult  is  guided  and  controlled  in  his  actions.  Mey- 
nert  has  charmingly  depicted  the  egotism  of  the  child,  and  the  constant 
struggle  of  the  healthy  individual  to  rid  himself  of  the  ideas  of  self  until  he 


520  THE  NERVOUS  DISEASES   OF  CHILDREN. 

is  enabled  to  sacrifice  his  individuality  and  his  entire  being  for  the  good  of 
the  family,  the  state,  and  of  all  mankind.  The  child  for  a  long  time  remains 
upon  that  lower  plane  in  which  passion  and  the  gratification  of  desires  are 
the  mainspring  of  all  action. 

The  experience  of  centuries  has  taught  us  that  by  gradual  stages,  and 
through  the  influence  of  training  and  education,  the  selfish  views  of  the 
child  can  be  transformed  into  the  more  generous  views  of  the  adult.  This 
process  of  evolution  may  be  checked  by  disease ;  it  is  only  by  comparison 
with  the  views  and  attainments  of  children  of  the  same  age  that  we  can  es- 
tablish the  standard  to  which  every  child  should  conform. 

Insanity  in  childhood  is  relatively  rare.  According  to 
the  statistics  of  Hagen  (quoted  by  Spitzka)  only  one  in 
70,684  children  annually  becomes  insane,  excluding  those 
born  so.  Spitzka  states  that  "  over  four  per  cent,  of  3,244 
adult  recorded  private  cases  may  be  justly  regarded  as 
having  been  insane  in  childhood,  while  but  twelve  patients 
afflicted  with  infantile  forms  of  insanity  "  were  observed  by 
him  during  their  infancy.  From  this  it  is  evident  that  we 
must  not  only  regard  the  full-fledged  insanities  of  early 
life,  but  also  those  morbid  conditions  in  early  years  which 
foreshadow  the  outbreak  of  mental  disease  in  later  years. 

The  psychoses  are  not  developed  in  very  young  infants, 
although  Greding  reports  a  child  that  was  a  "  raving 
maniac  "  at  birth.  The  story  must  be  taken  with  a  grain  of 
salt.  Rush  has  noted  the  occurrence  of  "  mania "  at  the 
age  of  two  years,  and  Sinkler  has  observed  it  in  children 
three  years  old. 

After  the  period  of  first  dentition  is  past,  faulty  psychic 
inheritance  may  become  noticeable ;  but  the  age  of  puberty 
is  the  one  fraught  with  greatest  danger.  The  author  had 
under  observation  various  members  of  a  family,  all  of  whom 
(two  boys  and  three  girls)  have  developed  mental  disease 
between  the  ages  of  twelve  and  fifteen  years.  All  classes 
of  the  socia1  body  seem  to  be  equally  prone  to  mental  dis- 
ease ;  while  idiocy  is  unquestionably  more  frequent  in  the 
poorer  classes  than  among  the  well-to-do. 

Etiology. — Heredity  is  the  potent  factor  in  the  cau- 
sation of  juvenile  and  adult  insanity.  There  is  always  the 
danger  of  over-estimating  the  importance  of  any  one  factor, 
but  the  daily  experience  of  alienists  and  neurologists  helps 
to  enforce  this  belief.     The  importance  of  heredity  is  im- 


INSANITY.  521 

pressed  upon  us,  too,  by  the  different  course  of  acquired 
insanity  in  persons  with  or  without  an  hereditary  taint. 
Psychic  disorders  constitute,  as  a  rule,  the  last  in  a  series 
of  degenerative  diseases ;  neurasthenia,  chorea,  epilepsy  in 
the  parent  may  lead  to  insanity  or  idiocy  in  the  child.  By 
way  of  exception  healthy  individuals  may  emanate  from 
families  with  a  distinct  neurotic  or  psychic  taint.  Mental 
derangement  in  children  is  ordinarily  developed,  if  one  or 
both  parents  have  been  insane,  if  one  or  both  have  been 
afflicted  with  a  severe  neurosis,  if  one  or  both  have  been 
addicted  to  alcohol,  and  above  all,  if  the  parents  are  related, 
and  belong  to  a  family  in  which  nervous  or  mental  disease 
has  been  of  common  occurrence.  Individuals  of  different 
but  tainted  families  will  also  be  liable  to  generate  insane 
children,  or  such  as  show  distinct  stigmata  of  degeneration 
(deformities  of  the  skull,  of  the  ears,  gothic  palate,  stunted 
growth,  abnormal  sexual  appetite,  early  masturbation,  and 
the  like).  Closely  allied  to  the  hereditary  forms  of  juvenile 
insanity  are  those  due  to  traumatism  during  birth.  Meyer 
has  insisted  on  this.  The  author  has  seen  innumerable 
cases  of  idiocy  due  to  this  cause,  but  not  a  single  instance 
of  any  other  form  of  mental  derangement. 

Arrested  physical  growth  is  often  associated  with  men- 
tal impairment  due  to  hereditary  causes.  A  girl,  aged 
twenty,  whom  I  have  seen  in  repeated  maniacal  attacks, 
is  stunted  in  growth,  being  only  as  tall  as  a  child  of 
twelve,  without  any  sign  of  mammary  development  or  of 
menstruation.  The  girl  stutters  a  little,  but  is  fairly  bright 
in  the  interval  between  the  maniacal  attacks. 

Among  the  causes  of  insanity  acquired  in  early  child- 
hood, the  chief  one  is  beyond  doubt  traumatism  to  the  skull. 
Emminghaus  has  found  this  factor  in  fourteen  of  one  hun- 
dred and  three  cases,  not  including  those  in  which  the  injury 
was  followed  by  epilepsy  and  mental  derangement.  Loss 
of  memory  and  complete  dementia,  or  maniacal  excitement, 
are  the  forms  of  insanity  commonly  developed  from  this 
cause.  The  occurrence  of  idiocy  after  relatively  slight  in- 
juries need  not  be  insisted  on  in  this  connection.  Menin- 
gitis early  in  life,  ear  disease,  insolation,  epilepsy,  hysteria, 
chorea,  severe  emotional  excitement,  grief,  shame,  disease  of 


522  THE   NERVOUS  DISEASES    OF   CHILDREN. 

the  heart,  intestinal  parasites,  acute  infectious  diseases  are 
the  conditions  which  predispose  to  the  development  of  in- 
sanity. To  these  might  be  added  excessive  work  at  school, 
maltreatment  at  home,  starvation,  and  cruel  punishments. 

Masturbation  is  another  and  a  potent  cause.  The  impor- 
tance of  this  factor  has  been  underrated  by  some  ;  it  can 
scarcely  be  overrated.  There  is  a  just  difference  of  opinion, 
however,  as  to  the  influence  it  exerts  in  generating  mental 
disease.  Many  claim  that  it  is  often  the  early  symptom  of 
an  insane  condition.  There  can  be  no  doubt  of  this,  but  it 
is  equally  certain  that  the  continuance  of  the  habit  brings 
about  a  rapid  deterioration  of  the  individual's  mental  state. 
It  is  often  acquired  by  imitation,  and  quite  as  often  it  would 
seem  to  be  the  result  of  natural  tendencies.  In  the  former 
class  the  practice  may  occasionally  be  checked  ;  in  the  lat- 
ter, the  habit  is  continued,  both  in  boys  and  girls,  in  spite 
of  the  most  persistent  watching.  The  more  stubborn  cases 
are  generally  those  characterized  by  other  distinct  signs  of 
degeneration.  The  evil  effects  of  the  vice  become  most 
pronounced  at  the  time  when  the  individual  is  subjected  to 
the  first  serious  strain.  Several  of  such  young  patients 
have  done  well  at  school,  have  passed  their  examinations 
successfully,  but  have  broken  down  completely  in  the  firrt 
competition  with  boys  of  mature  and  normal  intellects. 

FORMS   OF    INSANITY. 

A  detailed  account  of  all  the  symptoms  of  insanity 
would  far  exceed  the  limits  of  this  treatise.  By  combining 
adiscussionof  the  symptoms  with  a  description  of  the  types 
of  mental  derangement,  as  they  occur  in  early  life,  we  shall 
be  able  to  give  a  sufficient  survey  of  the  entire  subject.  It 
is  best  to  begin  with  those  symptoms  and  conditions  which 
are  on  the  border-line  between  sanity  and  insanity. 

Imperative  Concepts.— The  orderly  sequence  of  thought 
in  the  child  and  in  the  adult  may  be  interrupted  by  foreign 
and  recurrent  concepts,  which  obtrude  themselves  upon 
the  individual's  consciousness,  which  annoy  him  and  tyran- 
nize over  him,  but  of  which  he  is  not  able,  at  least  for  a 
time,  to  rid  himself. 


INSANITY.  523 

These  imperative  or  insistent  concepts  are  very  common  in 
childhood  ;  at  times  they  disappear  without  leaving  any 
trace  of  mental  defect  behind  them  ;  in  some  children  they 
represent  the  first  stages  of  chronic  forms  of  insanity. 
Imperative  conceptions  may  lead  to  imperative  actions,  to 
morbid  fears,  and  morbid  impulses. 

Many  normal  persons  have  passed  through  physiological  states  akin  to 
these  imperative  concepts.  On  going  to  bed  the  thought  arises  suddenly 
that  the  light  has  not  been  properly  turned  down,  that  the  gas  might  be 
escaping.  They  assure  themselves  that  all  is  well,  and  after  a  few  minutes 
the  same  fear  or  concept  comes  upon  them  again.  The  person  so  troubled 
may  attempt  to  allay  his  fears  by  methods  which  ordinarily  carry  conviction, 
but  in  spite  of  himself  the  same  insistent  idea  annoys  him.  Returning  to 
one's  door  again  and  again  to  make  sure  that  it  is  properly  locked  ;  the  an- 
noying thought  that  the  letter  which  has  just  been  mailed  was  not  properly 
signed  or  sealed ;  such  are  some  of  the  imperative  concepts  of  daily  life. 
The  sight  of  a  knife  may  suggest  the  harm  that  can  be  done  with  it ;  a  per- 
son standing  on  a  precipice  or  on  a  high  tower  has  a  feeling  as  though  he 
would  be  dragged  into  the  depths  below.  The  child,  after  it  has  passed  the 
first  five  or  six  years  of  life,  may  have  similar  experiences.  It  is  often  tort- 
ured by  these  insistent  concepts.  Some  pupils  at  school  have  to  do  every- 
thing over  and  over  again  ;  they  never  finish  a  sum  in  arithmetic  as  quickly  as 
others  because  the  doubt  arises  whether  they  have  done  it  properly,  and  they 
are  impelled  to  do  everything  a  definite  number  of  times  (arithmomania).  As 
a  rule,  they  can  assign  no  reason  for  this.  A  little  patient  would  never  ad- 
dress any  one  without  repeating  the  name  twice  ;  he  was  conscious  of  this 
peculiarity,  was  finally  induced  to  repeat  the  name  a  second  time  to  himself 
in  such  a  low  tone  of  voice  that  no  one  could  hear  it,  and  gradually  lost  the 
habit. 

Another  lad,  well  known  to  the  author,  would  do  everything  three  times 
for  fear  of  having  done  it  twice.  He  would  take  three  steps  at  a  time  in  going 
upstairs ;  if  there  were  but  two  he  would  jump  back  from  the  first  and  then 
walk  up  the  two,  so  as  to  make  three  in  all ;  before  going  to  bed  he  touched 
the  floor  three  times  ;  as  soon  as  he  was  in  bed  he  would  get  up  again  to 
make  sure  that  he  did  not  touch  it  twice.  These  concepts  and  actions  were 
imperative,  but  they  wrere  developed  from  a  fear  that  was  engendered  in  him 
at  the  time  of  his  father's  death  that  his  mother  might  be  the  second  one  to 
die.  This  caused  him  to  avoid  "  two  "  in  every  way.  After  a  number  of 
years  the  imperative  concepts  were  overcome. 

Closely  allied  to  these  conditions  are  the  fears  of  con- 
tamination (the  mysophobia  of  Hammond  ;  delire  du 
toucher  of  Legrand  du  Saulle).  I  have  known  several 
children  who  would  not  touch  the  knob  of  a  door,  or  take 


524  THE  NERVOUS  DISEASES   OF   CHILDREN. 

anything  out  of  other  people's  hands.  One  child  would 
not  be  washed  because  the  water  flowed  through  pipes  and 
the  pipes  were  not  clean.  The  fear  of  open  places  (agora- 
phobia of  Westphal)  occurs  in  children,  though  not  nearly 
so  often  as  in  adults.  Some  years  ago  the  author  treated  a 
young  girl,  twelve  years  of  age,  who  could  not  be  induced 
to  come  to  his  office  because  she  would  have  to  cross  a 
small  hill  over  a  tunnel ;  she  was  certain  that  she  could 
never  get  across.  These  fears  may  increase  in  such  num- 
ber as  to  give  rise  to  what  Mills  calls  pantophobia. 

Children  as  well  as  adults  are  troubled  by  insistent  ques- 
tions— generally  abstract  ones — which  interrupt  their  trains 
of  thought.  Why  is  the  sky  blue,  the  water  wet,  the  ball 
round  ?  Occasionally  they  soar  into  the  field  of  religion. 
Why  is  God  just?  and  so  on. 

All  these  conditions  may  be  of  slight  significance  unless 
they  lead  to  imperative  acts  of  violence  (cutting  a  child's 
throat  at  the  sight  of  a  knife,  etc.) ;  or  unless  the  effort  to 
suppress  these  morbid  concepts  and  impulses  produces  great 
excitement.  A  little  patient  of  mine,  years  ago,  was  so 
annoyed  that  he  would  every  now  and  then  pass  into  a 
maniacal  state  or  entertain  suicidal  ideas.  Westphal  was  of 
the  opinion  that  these  imperative  conceptions  were  never 
developed  into  delusions  ;  this  is  true  in  general,  but  they 
are  often  present  in  persons  who  develop  systematized 
delusions  suggested  by  the  contents  of  some  one  imperative 
conception.  Imperative  concepts  and  impulses  are  very 
apt  to  occur  in  children  who  have  passed  through  some  ex- 
hausting disease,  or  some  intense  emotional  excitement.  As 
children  grow  older  they  may  learn  to  disregard  annoying 
concepts,  and  thus  rid  themselves  of  them. 

Cerebral  Neurasthenia.  —  Nervous  exhaustion,  or 
neurasthenia,  is,  on  the  whole,  so  rare  in  children  that  we 
have  not  devoted  any  special  chapter  to  the  discussion  of 
this  disease.  The  causes  which  give  rise  to  neurasthenia  in 
the  adult  are  far  less  potent  in  childhood.  Excitement, 
over-work,  cares  in  early  years,  are  more  apt  to  produce 
hysterical  and  choreic  conditions  than  neurasthenia ;  occa- 
sionally, however,  the  cerebral  type  is  developed  in  young 
persons  between  the  ages  of  ten  and  fifteen  years  or  older. 


INSANITY.  525 

The  chief  symptoms  are,  inability  to  concentrate  the  at  ten- 
tion  upon  any  special  work,  or  excessive  restlessness  follow- 
ing upon  such  an  effort.  A  feeling  of  pressure  upon  the 
top  of  the  head,  a  dragging  sensation  over  the  nape  of  the 
neck  and  the  upper  portion  of  the  spine,  general  irritability, 
a  morose  disposition,  sleeplessness  and  a  feeling  of  fatigue, 
a  slight  increase  in  the  reflexes,  sensations  of  heat  and  cold 
complete  the  details  of  this  condition. 

Cerebral  neurasthenia  occurs  mainly  in  the  children  of 
hysterical  or  excessively  neurotic  parents,  and  in  those  who 
come  of  healthy  parentage  but  are  pushed  inordinately  in 
their  work  at  school  to  satisfy  the  demands  of  ambitious 
parents.  It  has  been  the  custom  to  attach  the  entire  blame 
to  present  educational  methods,  but  the  chief  fault  lies  with 
the  father  or  mother  who  cannot  recognize  the  child's  in- 
ability to  cope  with  other  children  of  the  same  age.  Boys 
or  girls  who  recognize  that  they  are  at  a  disadvantage  in 
competition  with  others  become  discouraged  and  moody, 
and  often  develop  a  condition  of  melancholy  depression. 

Some  years  since,  a  young  girl,  aged  thirteen,  was  brought  to  me,  who  had 
just  entered  upon  the  work  of  a  high-school  in  which  an  older  sister  had 
graduated  with  high  honors.  The  patient  was  told  by  her  mother  that  she 
would  be  a  discredit  to  her  family  unless  she  did  at  least  as  well  as  the  older 
sister  had  done.  The  child  made  a  strenuous  effort  to  come  up  to  these 
expectations,  but  the  result  was  evident  on  examination.  The  child  com- 
plained of  intense  headaches,  frontal  and  vertical,  of  a  feeling  as  though 
she  were  being  crushed  under  a  weight.  She  looked  pale  and  haggard 
in  consequence  of  the  loss  of  sleep.  Her  appetite  had  left  her,  and  for  a  few 
days  before  she  was  examined  was  either  in  a  drowsy  condition  or  else 
would  cry  and  complain  of  her  misfortune.  She  knew  that  she  could  not 
possibly  do  satisfactory  work  at  school,  and  had  developed  the  idea  that  she 
was  thoroughly  unworthy  of  the  care  which  her  parents  had  bestowred  upon 
her. 

The  child  was  taken  out  of  school  at  once,  sent  to  some  relatives  in  the 
country,  and  was  told  that  she  would  get  entirely  well  provided  she  would 
not  think  of  school,  or  anything  connected  with  school  matters,  for  a  period  of 
several  months.  The  parents  were  also  instructed  never  again  to  force  the 
child's  work,  or  to  foster  any  inordinate  ambition  in  her.  The  older  sister 
was  a  bright,  strong,  well-developed  girl,  who  could  in  all  probability  have 
stood  any  amount  of  work  without  showing  signs  of  fatigue.  The  younger 
child  was  less  well  developed  physically,  and  presented  the  remnants  of 
former  internal  hydrocephalus   (a  bulging  forehead  and  very  large  transverse 


526  THE   NERVOUS  DISEASES    OF   CHILDREN. 

occipital  diameter).  Had  the  parents  been  allowed  to  continue  in  their  foolish 
course  much  longer,  this  child,  who  presented  on  the  whole  none  but  neuras- 
thenic symptoms,  would  unquestionably  have  developed  a  serious  form  of 
psychosis. 

The  course  of  cerebral  neurasthenia  may  cover  a  period 
of  several  weeks  or  months,  according  to  the  time  at  which 
a  radical  effort  is  begun  to  check  the  development  of  the 
disease ;  but  the  affection  is  an  eminently  curable  one.  The 
condition  should  be  treated  very  much  as  is  the  same  disease 
in  the  adult.  The  most  important  point  is  to  give  body  and 
mind  complete  rest,  to  take  the  child  from  school  and  to 
prohibit  all  mental  fatigue  either  in  the  way  of  school  or  of 
home  studies.  If  a  proper  arrangement  can  be  made,  it 
is  best  to  place  the  child  under  care  of  a  sensible  nurse,  and 
subject  it  to  the  rest-cure.  A  separation  from  hysterical 
and  irritating  relatives  is  absolutely  essential.  Careful  feed- 
ing and  the  ordinary  tonics — above  all,  iron,  arsenic,  and 
small  doses  of  strychnia — will  help  to  bring  about  a  rapid 
improvement.  After  four  to  six  weeks  the  child  should  be 
allowed  to  roam  about  in  the  country,  and  its  mind  should 
at  least  for  a  year  or  more  be  kept  entirely  free  from  every 
sense  of  duty  and  obligation. 

Hypochondriasis  is  often  associated  with  neurasthenia, 
and  occasionally  it  is  a  primary  affection.  The  fear  of  in- 
sanity is  not  as  pronounced  as  in  adult  neurasthenics,  but 
the  fear  of  death  is  common.  The  uncomfortable  sensations 
in  the  head  frighten  the  child,  and  soon  it  takes  notice  of 
every  little  symptom  to  which  other  children  would  pay 
no  attention.  In  such  introspection  children  are  encour- 
aged by  over-anxious  mothers.  Like  the  adult,  the  young 
hypochondriac  hears  his  heart  palpitate,  takes  notice  of  the 
rumbling  of  his  bowels,  watches  his  skin  most  carefully, 
and  exaggerates  the  importance  of  every  pimple  that  ap- 
pears upon  it.  At  the  age  of  puberty  a  boy's  attention  is 
rivetted  easily  upon  the  sexual  organs.  He  may  be  fright- 
ened by  the  occurrence  of  erections  or  by  the  difference  in 
the  size  of  the  testicles.  A  bright  lad,  aged  fourteen,  whom 
I  treated  for  a  long  time,  and  who  came  of  a  highly  neu- 
rotic family,  was  at  great  pains  to  prove  to  me  that  his 
testicles  were  "  detached  "  from  the  rest  of  his  body,  be- 


INSANITY.  527 

cause  he  could  move  them  about  freely.  Early  masturbation 
intensifies  such  fears.  Westphal  reports  the  history  of  a 
young-  hypochondriac  who  complained  of  abnormal  sen- 
sations in  the  head,  in  the  feet,  in  the  abdomen  ;  his  tears 
had  been  dried  up,  and  all  the  mucus  in  the  lungs  had  been 
expectorated.  The  boy's  hypochondriacal  mood  was  inter- 
rupted by  a  mild  attack  of  maniacal  excitement.  The  prog- 
nosis of  hypochondriasis  in  the  young  is  entirely  favorable 
unless  it  is  based  upon  a  marked  hereditary  taint.  The  cure 
of  the  condition  lies  in  sensible  management  of  the  youth's 
mode  of  life,  in  diverting  his  mind  from  himself  by  moder- 
ate indulgence  in  out-door  sports,  by  providing  him  with 
the  companionship  of  sober-minded  lads  of  the  same  age. 
I  have  known  boys  suffering  from  sexual  hypochondriasis 
to  be  forced  by  parents  into  early  intercourse  with  the 
opposite  sex.     The  procedure  is  invariably  harmful. 

The  preceding  forms  of  mental  derangement  are  less 
serious  than  those  which  we  must  now  consider. 

Mania. — The  term  "  mania  "  denotes  a  form  of  mental 
disease  in  which  there  is  a  marked  acceleration  of  all  cere- 
bral and  physical  functions,  generally  associated  with  a 
feeling  of  well-being.  In  children  this  condition  is  rela- 
tively rare ;  it  is  often  confounded  with  a  temporary  active 
delirium,  which  comes  on  after  the  ordinary  febrile  diseases 
of  childhood.  In  true  mania  restraint  is  entirely  removed  ; 
the  patient's  ability  and  desire  to  do  anything  and  every, 
thing  he  chooses  knows  no  bounds.  Thoughts  and  impulses 
follow  rapidly  upon  one  another.  Emminghaus  has  well 
said  that  an  exalted  mood,  a  rapid  succession  of  incomplete 
thoughts,  unbounded  desire  to  make  everything  it  sees  its 
own,  are  characteristic  of  the  healthy  child.  But  the  normal 
child,  under  the  restraining  influences  of  education  and  train- 
ing, soon  learns  to  curb  its  desires  and  to  develop  an  orderly 
train  of  thought.  The  young  maniac  has  apparently  cast 
aside  all  restraint  and  gives  himself  up  completely  to  his 
passions  and  his  impulses.  His  incessant  activity  leads  him 
to  tear  and  destroy  everything  within  his  reach  ;  he  is  cruel 
to  others  and  does  not  hesitate  to  inflict  injury  upon  him- 
self. There  is  no  trace  of  a  sense  of  decency  or  propriety, 
even  young  children  using  the  foulest  language.     Immature 


528  THE  NERVOUS  DISEASES   OF   CHILDREN. 

thoughts  follow  rapidly  upon  one  another  ;  everything  the 
maniac  hears  amuses  him;  a  word  suggests  another  that 
rhymes  with  it ;  his  answers  are  often  quick,  sometimes 
bright ;  but  they  are  flashes  merely  and  do  not  denote  real 
intelligence. 

The  excessive  restlessness  and  the  rapid  flight  of  ideas 
are  not  followed  by  a  feeling  of  fatigue,  which  would  be 
natural  in  a  normal  person.  Loss  of  appetite  and  of  sleep 
contribute  to  the  exhaustion  which  is  developed  if  the  con- 
dition lasts  for  a  number  of  weeks  or  months. 

The  symptoms  of  mania  come  on  in  an  insidious 
fashion,  or  may  be  preceded  by  a  period  of  depression.  At 
first  the  greater  liveliness  and  activity  of  the  child  are  sup- 
posed to  be  a  mere  exaggeration  of  its  normal  state  ;  by 
degrees  the  increase  of  all  the  symptoms  points  to  a  morbid 
state  ;  after  the  acme  of  the  disease  has  been  reached  the 
condition  remains  unchanged  for  some  weeks,  and  then  the 
excitement  gradually  diminishes.  A  return  of  sleep  is,  as  a 
rule,  the  first  favorable  sign  pointing  toward  recovery.  Re- 
current mania  (several  attacks  and  remissions)  has  been 
described.  The  exact  duration  of  a  maniacal  attack  may 
vary  between  five  months  and  a  year,  but  the  state  of  in- 
tense excitement  is,  as  a  rule,  much  shorter. 

Among  the  etiological  factors  the  acute  febrile  condi- 
tions and  severe  strain  or  emotional  excitement  are  the 
most  potent.  The  period  of  beginning  menstruation  is 
fraught  with  the  greatest  danger.  The  only  cases  of  true 
mania  which  I  have  seen  were  in  girls  at  this  period,  and  in 
a  boy,  aged  fifteen.  One  of  the  girls  had  passed  through 
a  series  of  examinations  at  school  with  great  credit  to  her- 
self. On  the  morning  following  the  last  examination  she 
refused  to  get  out  of  bed,  would  not  take  her  food,  and 
would  not  allow  herself  to  be  washed.  To  all  questions  why 
she  would  not  do  as  asked,  she  mumbled  a  few  inaudible 
words ;  soon  she  became  wholly  silent,  and  for  a  period  of  four 
weeks  she  lay  in  an  absolutely  stuporous  condition,  passing 
urine  and  fasces  into  the  bed,  and  refusing  to  take  nourish- 
ment. From  this  condition  she  passed  quite  suddenly  into 
one  of  most  violent  mania,  in  which  she  tore  everything 
that  she  could  lay  hands  on,  sang  and  cursed  all  day  long, 


INSANITY.  529 

spat  at  every  one,  tore  her  own  clothes  into  shreds,  and 
would  expose  her  person  before  every  one.  (In  health  she 
was  a  sweet  well-behaved  child.)  The  state  of  maniacal 
excitement  lasted  four  months,  during  which  time  she  lost  a 
great  deal  of  flesh.  Six  months  after  the  first  symptoms  had 
appeared,  menstruation  set  in,  and  from  that  time  onward 
a  rapid  and  complete  recovery  ensued.  In  other  cases  I 
have  observed  a  development  of  mania  after  the  cessation 
of  the  menses  that  had  appeared  a  few  times.  A  close 
relation  between  the  menstrual  flow  and  these  psychic  con- 
ditions cannot  be  doubted. 

While  the  prognosis  is  favorable,  careful  treatment  is 
necessary  in  all  cases.  ,  These  children  must  be  guarded  by 
competent  nurses ;  mechanical  restraint  cannot  always  be 
avoided,  but  it  should  not  be  more  forcible  than  necessary. 
Such  patients  are  generally  fit  subjects  for  an  asylum,  and 
often  do  better  there  than  at  home,  where  greater  restraint 
has  to  be  applied.  The  author  has  treated  such  patients, 
however,  in  their  homes  by  placing  them  in  large,  well-venti- 
lated rooms,  away  from  the  rest  of  the  family  and  under 
the  charge  of  two  or  more  nurses. 

In  home  and  asylum  treatment,  the  hydrobromate  of 
hyoscin  (one  two-hundredth  to  one  one-hundredth  grain 
three  times  daily),  given  by  the  mouth  or  hypodermically, 
is  a  valuable  drug.  In  addition,  sulfonal,  trional  or  veronal, 
in  moderate  doses,  should  be  given  at  night.  Bromides  are 
entirely  useless,  and  opiates  of  little  benefit.  Prolonged  full 
baths,  followed  by  slightly  cooler  ablutions  of  the  spine 
tend  to  calm  the  excitement,  and  sometimes  help  to  induce 
sleep  when  all  other  measures  fail. 

Melancholia  represents  a  distinct  form  of  insanity 
which  has  been  observed  in  children,  particularly  between 
the  ages  of  eight  and  fifteen  years.  It  is  not  to  be  con- 
founded with  simple  melancholy  depression,  which  may 
accompany  many  other  morbid  mental  states.  It  is  as  nat- 
ural for  a  lunatic  who  supposes  himself  to  be  the  victim  of 
persecution,  or  a  target  for  the  raillery  of  others,  to  be  de- 
pressed and  melancholy  as  it  would  be  for  a  sane  person 
who  found  that  all  his  efforts  to  succeed  were  in  vain,  and 
that  everything  and  everybody  were  against  him. 


530  THE  NERVOUS  DISEASES   OF   CHILDREN. 

In  true  melancholia  the  child  is  depressed  without 
cause.  Experiences  which  would  please  others  make  no 
impression  upon  its  saddened  mind.  It  does  not  care  to 
play  with  other  children,  whose  frolicsome  ways  are  a 
source  of  annoyance.  Games,  books,  theatres,  have  no 
charm  for  the  melancholy  child ;  it  seeks  seclusion,  sits  in 
a  corner  by  itself  all  day  long ;  will  not  speak  spontane- 
ously, and,  if  spoken  to,  either  does  not  answer  at  all  or 
replies  in  monosyllables  and  with  much  hesitation.  No 
reason  for  its  qaoodiness  is  given ;  after  much  questioning 
it  may  say  that  it  feels  sad,  but  does  not  know  why.  To 
the  questions  put  to  a  little  patient  I  received  the  answer, 
"  I  know,  I  know,"  accompanied  by  a  nodding  of  the  head. 
As  it  was  recovering,  the  child  stated  that  she  knew  every- 
one was  against  her ;  that  her  parents  did  not  care  for  her ; 
that  she  was  to  be  punished  because  she  had  not  loved 
them  enough,  and  because  she  was  so  ugly  (as  a  matter  of 
fact  she  was  good-looking).  As  this  same  child  was  taken 
to  the  country  to  hasten  convalescence,  she  said :  "  I  know, 
I  know,  you  will  burn  me  to  ashes." 

In  contradiction  to  the  acceleration  of  all  mental  proc- 
esses in  mania  there  is  a  distinct  inhibition,  a  "  slowing 
up  "  of  all  mental  and  physical  processes  in  melancholia. 
Loss  of  appetite,  restless  sleep,  slowness  of  all  muscular 
efforts,  obstinate  constipation  are  characteristic  features  of 
this  disorder.  The  child  may  be  able  to  reason  a  little  re- 
garding its  unfortunate  condition,  and  self-accusations  are 
abundant.  It  accuses  itself  of  unkindness  toward  others, 
of  want  of  respect  and  love  for  its  elders ;  if  it  has  had  any 
religious  training  it  develops  the  ideas  of  the  "  unpardon- 
able sin,"  and  of  lack  of  devotion  to  God — ideas  which 
play  a  prominent  part  in  the  melancholy  of  adults.  Hallu- 
cinations in  keeping  with  the  depressed  mood,  the  sight 
of  the  devil,  of  a  cruel  teacher  armed  with  all  sorts  of 
weapons  of  torture,  may  increase  the  depression,  or  else 
lead  to  a  condition  of  excitement  or  frenzy  (melancholia 
agitata).  Unlike  the  maniac,  the  melancholy  patient  di- 
rects his  frenzied  impulses  toward  his  own  person.  Self- 
mutilation  is  common.  A  young  girl,  aged  thirteen,  had 
to    be    restrained    because    she    would    insist    upon    tear- 


I. YS  AMITY. 


53' 


ing    out   her   hair   and    attempted    to   cut   deep   into    her 
skin. 

Suicide  of  children  is  frequently  the  result  of  melancholia.  Like  the 
adult,  the  child  seeks  to  put  an  end  to  a  life  that  is  so  full  of  trouble  and 
misery.  Statistics  as  to  the  frequency  of  suicide  in  children  are  rather  unsatis- 
factory ;  Emminghaus  quotes  those  of  Morselli  that  show  the  number  to  be 
greatest  in  Denmark  and  Prussia,  and  smallest  in  Belgium  and  Italy.  The 
author  has  seen  no  statistics  bearing  upon  America.  In  the  majority  of 
cases  some  mental  disease  is  the  cause  of  suicide  in  the  young,  and  none  is 
more  frequent  than  melancholy.  Some  suicidal  attempts  are  due  to  trifling 
causes — fear  of  punishment,  chagrin  over  an  unmerited  rebuke,  etc. 

Melancholia  with  stupor  (melancholia  attonita)  is  a  more 
extreme  form,  which  is  either  developed  primarily  or  fol- 
lows upon  a  condition  of  simple  melancholy.  In  this  form 
the  child  lies  in  bed  absolutely  motionless,  takes  no  notice 
of  its  surrounding's,  and  cannnot  be  induced  to  smile  or  to 
say  a  single  word.  It  reacts  but  feebly  to  every  form  of 
cutaneous  stimulation  ;  it  will  to  a  certain  degree  tolerate 
pain  rather  than  move.  If  passive  movement  is  attempted, 
the  limbs  will  retain  the  position  given  them,  or  the  child 
offers  considerable  resistance.  Urine  and  faeces  are  passed 
into  the  bed.  During  this  condition  of  stupor  disagreeable 
hallucinations  and  terrorizing  delusions  add  to  the  child's 
misery. 

In  the  author's  experience  melancholia  is  the  most  fre- 
quent form  of  mental  derangement  in  childhood.  Its 
course  varies  very  much.  Some  patients  get  well  in  a  few 
months,  others  do  not  recover  for  more  than  a  year.  In  a 
number  of  instances  a  condition  of  mania  follows  upon  the 
period  of  depression.  The  prognosis  is  favorable  as  regards 
ultimate  recovery ;  it  is  certain  that  sixty  per  cent,  of 
young  melancholy  subjects  get  well.  As  a  rule,  the  pros- 
pects of  early  recovery  are  better  in  the  agitated  form,  and 
less  bright  in  the  stuporous  form.  Melancholy  may  ter- 
minate in  dementia,  or  in  death  from  exhaustion  or  from 
suicide.* 

The  treatment  of  melancholia  is  very  simple.     One  or 

*  The  author  has  not  entered  upon  an  enumeration  of  the  morbid  anatomy  of 
melancholia  or  mania,  as  there  is  nothing  but  pure  hypothesis  to  proceed  upon.  The 
vascular  theories  of  Meynert  have  not  been  corroborated  by  others. 


532  THE  NERVOUS  DISEASES   OF   CHILDREN. 

more  competent  nurses  must  be  deputed  to  watch  over  the 
child  and  protect  it  against  all  harm.  It  must  be  fed  care- 
fully with  a  spoon,  and  will  do  best  on  milk,  some  cereals, 
scraped  meat,  and  eggs.  The  stomach-tube  should  not  be 
resorted  to  unless  absolutely  necessary.  Opium  in  small 
doses,  or  hyoscyamia  in  the  agitated  forms,  are  the  most  use- 
ful drugs  ;  both  can  be  given  hypodermically  if  necessary, 
but  they  should  be  discontinued  as  soon  as  practicable.  Sul- 
fonal,  chloralamid,  or  trional  will  help  to  induce  sleep  and  to 
reduce  mental  excitement.  As  in  cases  of  mania,  a  warm, 
full  bath  is  a  valuable  aid  in  treatment. 

There  is  no  sufficient  reason  to  remove  such  children 
from  comfortable  homes,  if  isolation  at  home  is  possible  ; 
but  if  the  environment  of  the  child  is  an  unfavorable  one, 
the  sooner  it  is  taken  to  an  asylum  the  better  it  will  be. 

Periodic  and  Circular  Insanity  are  very  exceptional 
occurrences  in  children.  The  youngest  patient  of  this  class 
the  author  has  seen  was  a  boy,  aged  eighteen,  and  all  of  the 
patients  described  by  Krafft-Ebing,  Jacobi,  Kelp,  and  others 
have  been  near  or  beyond  the  age  of  puberty.  Periodic 
insanity  consists  of  successive  attacks  of  mania  or  of  melan- 
cholia, followed  by  a  lucid  interval  of  months  or  years,  and 
then  a  recurrence  of  the  same  conditions.  Circular  insanity 
is  closely  allied  to  this  form.  As  described  by  Krafft-Ebing 
it  is  distinguished  from  the  ordinary  periodic  insanity  by 
an  alternation  of  maniacal  and  melancholy  stages  followed 
by  a  lucid  interval,  and  then  a  recurrence  of  derangement 
in  the  same  or  the  reverse  order  as  before.  The  diagnosis 
of  circular  insanity  can  be  suspected,  but  not  made,  until 
the  patient  has  passed  through  several  cycles. 

The  duration  of  each  cycle  may  vary  from  several  days 
to  several  weeks ;  but  in  the  author's  experience  as  the  dis- 
ease progresses  the  lucid  periods  grow  shorter  and  shorter. 
The  perodic  and  circular  forms  of  insanity  are  of  the  herid- 
itary  degenerative  type,  and  the  prognosis  in  them  is  far 
less  favorable  than  it  would  be  in  ordinary  mania  or  melan- 
cholia. During  the  maniacal  or  melancholy  state  the  treat- 
ment would  be  similar  to  that  described  for  each  con- 
dition. These  patients  are  dangerous  to  themselves  and 
their  surroundings,  as  they  pass  quickly  from  one  stage  to 


INSANITY.  533 

another.  As  soon  as  the  circular  and  periodic  character  of 
the  disease  is  established,  it  is  best  to  keep  such  children 
under  the  constant  observation  of  an  attendant,  or  else  to 
place  them  in  special  institutions. 

The  author  has  not  departed  from  the  above  account  as  given  in  the  first 
edition  of  this  book,  although  he  is  well  aware  of  the  changes  which  have 
been  brought  about  in  the  classification  of  early  mental  disorders  through  the 
writings  of  Kraepelin  and  his  followers,  who  have  substituted  for  periodic 
and  circular  insanity  the  conception  of  a  manic-depressive  form  of  insanity. 
With  the  exception  of  the  involution  melancholias,  Kraepelin  would  con- 
sider all  periodic  or  recurrent  forms  of  mania  and  melancholia  to  be  merely 
different  manifestations  of  a  single  psychosis,  viz.,  manic-depressive  insanity. 
Startling  and  revolutionary  as  Kraepelin's  doctrines  may  appear  to  be,  the 
writer's  ample  experience  would  lead  him  to  indorse  this  view,  at  least  in  part, 
but  he  must  also  insist  that  many  a  youthful  individual  may  pass  through 
periods  of  maniacal  excitement  or  of  depression  without  such  an  individual 
necessarily  being  a  victim  of  a  circular  or  manic-depressive  psychosis. 

Dementia  precox  :  Following  Kraepelin's  suggestions,  alienists  and 
neurologists  have  adopted  this  unfortunate  term,  designating  thereby  an  entire 
series  of  youthful  or  adolescent  forms  of  insanity  characterized  by  a  distinct 
tendency  to  progressive  deterioration  of  mind.  French  authors,  the  writer, 
Dercum,  and  others  have  been  at  some  pains  to  protest  against  the  too  liberal 
use  of  this  term  and  against  the  practice  of  labeling  almost  every  form  of 
youthful  insanity  as  a  dementia  prsecox,  for  many  of  these  patients  never 
develop  a  condition  of  dementia  and  should  be  spared  the  stigma  of  a  name 
that  implies  an  incurable  malady.  On  the  other  hand,  it  is  well  to  admit  that 
mental  enfeeblement  is  a  frequent  accompaniment  of  insanity  in  early  life. 

Dementia  Prascox  includes  three  distinct  forms,  each  one  of  which  may  be 
developed  at  the  age  of  puberty  or  even  earlier  :  (i)  Hebephrenia  ;  (2)  Kata- 
tonia ;  (3)  the  Paranoid  form  of  Dementia  PrEecox. 

Hebephrenia  as  described  by  Kahlbaum  and  Hecker  is  characterized  by  a 
period  of  depression,  with  hallucinations  and  delusions  of  a  corresponding 
type  ;  the  patients  are  often  conscious  of  their  altered  mental  state  and  recog- 
nize their  inability  to  vie  with  others  of  their  age  at  school  or  even  in  sport  j 
they  lack  the  power  of  concentration  and  of  attention.  Their  minds  are 
easily  diverted  from  the  subject  in  hand,  their  memory  and  judgment  become 
defective,  they  are  indifferent  to  family  and  friends.  Volubility  of  speech  may 
for  a  time  conceal  a  real  mental  defect.  The  patients  laugh  or  cry  without 
reason,  are  apt  to  repeat  high-sounding  phrases,  their  letters  are  verbose  and 
meaningless,  their  conduct  and  manners  become  affected,  silly,  theatric,  and 
their  moral  sense  may  be  more  or  less  impaired.  According  to  Kraepelin's  sta- 
tistics only  8  per  cent,  of  hebephrenic  patients  have  any  chances  of  recovery. 

Katatonia,  first  described  by  Kahlbaum,  is  characterized  by  states  of 
stupor  or  excitement  associated  with  conditions  of  "  negativism,"  of  muscular 
tension,  of  impulsiveness,  with  a  tendency  to  automatic  and  stereotyped  ac- 


534  THE  NERVOUS  DISEASES   OF   CHILDREN. 

tions,  and  with  automatic  repetition  of  words.  All  these  peculiar  mental 
conditions  lead  to  distinct  deterioration  within  a  reasonably  short  period  of 
time. 

The  paranoid  form  of  dementia  praecox  is  characterized  by  the  develop- 
ment of  an  entire  series  of  extravagant,  absurd  and  ever  changing  delusions, 
coming  on  frequently  with  an  initial  period  of  depression  and  leading  within 
a  few  years  to  a  condition  of  undoubted  dementia.  Its  onset  early  in  life  and 
the  rapid  development  of  a  dementia  will  help  to  distinguish  it  from  true 
paranoia. 

If  we  accept  the  Kraepelin  doctrines  in  whole  or  in  part,  the  prognosis  of 
these  forms  must  be  given  in  very  guarded  fashion.  But  there  seems,  to  the 
writer,  to  be  sufficient  doubt  in  many  of  these  cases  as  to  the  ultimate  out- 
come to  make  it  incumbent  upon  the  physician  to  urge  that  such  individuals 
be  separated  from  the  family  at  once,  that  they  be  given  for  a  time,  at  least, 
wholesome  employment  in  the  country  or  elsewhere,  and  that  they  should  not 
be  made  asylum  inmates  until  they  have  proved  either  that  they  are  incapable 
of  conducting  themselves  properly  when  at  large,  or  that  their  psychic  condi- 
tion is  such  that  they  need  institution  treatment  for  their  own  protection  and 
for  the  improvement  of  their  symptoms. 

Paranoia.* — Primary  insanity  is  a  degenerative  psy- 
chosis of  the  hereditary  order  characterized  by  hallucina- 
tions and  delusions.  The  latter  are  primary  symptoms  and 
not  secondary  to  the  exalted  or  depressed  mood,  as  in 
mania  and  melancholia.  The  delusions  or  "  fixed  ideas,"  be- 
come systematized,  and  dominate  the  mental  activity  of  the 
individual  to  such  a  degree  that  they  become  the  mainspring 
of  all  action.  The  paranoiac  is  not  amenable  to  reasoning  ; 
and  his  delusions  cannot  be  dislodged  by  argument,  as 
happens  with  the  temporary  delusions  of  the  sane.  He 
may  have  one  set  of  delusions  or  many.  The  persistence  of 
one  or  many  proves  that  the  entire  logical  apparatus  is  out 
of  gear.  It  is  wrong  to  claim  that  any  person  is  insane  on 
one  point  only  ;  he  may  show  his  insanity  in  one  direction 
chiefly,  but  his  mental  derangement  is  as  marked  as  though 
he  had  dozens  of  fixed  ideas. 

The  systematized  delusions  of  paranoia  may  be  divided 
into  two  great  groups  ;  first,  delusions  of  persecution  ;  and 
secondly,  delusions  of   grandeur  ;    the  latter  may  be  sub- 

*  The  writings  of  Snell,  Westphal,  Sander,  Krafft-Ebing,  and  Meynert,  have  con- 
tributed most  to  an  understanding  of  this  subject.  Spitzka  treats  the  subject  very 
lucidly  under  the  name  "  Monomania,''  preserving  an  old  term,  but  discarding  the  doo 
trine  of  the  "  Monomanias  "  which  did  so  much  harm  in  psychiatry. 


INSANITY.  535 

divided  into  the  religious,  the  political,  and  the  erotic  type. 
In  this  form  of  insanity,  with  delusions  of  persecution,  the 
patient  believes  himself  to  be  the  victim  of  circumstances. 
He  is  made  to  suffer  either  for  wrongs  he  has  committed 
or  for  the  envy  others  feel  toward  him.  He  has,  as  a  rule, 
been  morose  and  exclusive.  He  feels  that  he  is  being  ob- 
served by  others  ;  that  every  one  notices  a  peculiarity  in 
him  ;  that  others  can  read  and  control  his  thoughts  ;  that 
the  newspapers  direct  their  flings  at  him  ;  when  they  speak 
of  rascals,  of  thieves,  they  mean  him.  Before  long  he  hears 
the  voices  of  his  enemies  who  are  trying  to  ferret  out  his 
actions  ;  he  stops  up  the  key-holes  and  draws  the  blinds  of 
his  windows.  If  his  neighbors  cannot  get  rid  of  him  as 
speedily  as  they  wish,  they  put  poison  in  his  food,  which 
he  refuses  to  take.  He  supposes  himself  the  victim  of  the 
police,  of  socialists,  of  a  religious  sect,  who  will  endeavor 
to  influence  him  by  electricity,  through  the  telephone,  by 
hypnotizing  him,  or  by  forcing  him  to  inhale  all  sorts  of 
noxious  vapors. 

Paranoia  with  delusions  of  grandeur  includes  all  those 
who  imagine  themselves  destined  to  fulfil  some  special  mis- 
sion ;  the  political  reformers,  the  Guiteaus,  the  religious  fa- 
natics, the  presidents,  the  emperors  and  kings,  the  Goulds 
and  Vanderbilts  among  the  insane,  belong  to  this  class 
chiefly.  Guiteau,  descended  from  a  father  who  believed 
in  Mesmerism  and  in  free-love,  was  particularly  fond  of 
reading  on  religious  subjects  ;  he  masturbated  at  an  early 
age  and  entered  the  Oneida  Community  at  the  age  of  nine- 
teen ;  when  twenty-four  years  old  he  writes  to  his  father 
saying  that  he  was  employed  by  Jesus  Christ  &  Co.  Some 
paranoiacs  exhibit  signs  of  the  insane  neurosis  at  a  still 
earlier  day. 

The  children  who  are  exclusive,  who  never  care  to  play 
with  others,  who  pray  when  their  comrades  frolic  about 
— these  are  the  very  ones  who  develop  paranoia  or  the 
paranoid  form  of  dementia  prascox.  Moody,  queer,  and 
"cranky,"  they  go  along  well  enough  until  they  have  to  com- 
pete with  others  in  the  struggle  for  existence,  or  until  they 
are  overcome  by  some  severe  grief,  by  strong  emotion,  by 
political  or  religious  excitement  (the  election  campaigns,  the 


53^  THE  NERVOUS  DISEASES   OF   CHILDREN. 

revival  meetings,  and  the  like) ;  and  then  delusions,  which 
may  have  been  latent  for  a  long  time  come  to  the  fore- 
ground. The  paranoiac  comes  of  neurotic  stock,  in  which 
insanity,  hysteria,  epilepsy,  and  chronic  alcoholism  have 
been  common  occurrences. 

Not  all  children  who  are  morose  and  exclusive  turn  into 
paranoiacs,  but  it  is  well  to  look  with  suspicion  and  fear 
upon  youthful  prodigies,  who  discuss  philosophy,  or  work 
at  visionary  schemes  while  other  boys  are  engaged  in  sport. 
Children,  as  well  as  adults,  should  exhibit  a  modicum  of 
learning  and  of  virtues,  and  possibly  a  few  vices.* 

Emminghaus  describes  a  form  of  acute  paranoia  with 
hallucinations  which  is  said  to  occur  in  children  after  febrile 
attacks,  but  the  cases  he  quotes  are  not  very  convincing ; 
they  are  to  be  distinguished  from  ordinary  mania  by  the 
evidence  of  hallucinations  and  by  their  mode  of  onset. 

The  course  of  paranoia  is  eminently  chronic  ;  for  a  time 
slight  remissions  may  occur,  enabling  the  person  to  return 
to  some  regular  occupation.  He  may  be  able  to  keep  his 
delusions  in  abeyance  for  a  varying  period  of  time,  but  they 
will  come  to  the  front  in  the  end.  The  chronic  stage  is 
established  sooner  or  later,  in  which  he  is  entirely  con- 
trolled by  his  delusions,  and  is  a  fit  subject  for  asylum  treat- 
ment. Very  little  can  be  attempted  in  this  disease  in  the 
way  of  treatment,  but  if  the  first  traces  of  the  disorder  are 
observed  in  a  young  boy,  a  special  endeavor  should  be 
made  to  provide  him  with  healthful  surroundings,  to  di- 
vert his  mind  from  his  own  person,  and  to  arouse  his 
interest  in  those  things  which  are  the  reverse  of  those  he 
is  accustomed  to  brood  over.  Special  teachers,  and  tours 
to  foreign  lands  may  accomplish  something ;  but  disap- 
pointment to  parent,  teacher,  and  physician  is  the  most 
common  result. 

Moral  Insanity. — We  need  say  little  of  this  condition, 
which  has  given  rise  to  so  much  discussion.  It  is  generally 
conceded  that  a  lack  of  the  moral  sense  may  be  the  chief 
feature,  but  this  moral  imbecility  is  generally  associated 
with  a  defect  in  the  intellectual  sphere  ;  hence  many  of  the 

*  In  this  chapter  the  author  has  drawn  occasionally  upon  the  descriptions  given  by 
him  in  his  article  on  Insanity  and  Crime,  in  Hamilton's  System  of  Legal  Medicine. 


INSANITY  537 

subjects  of  moral  insanity  are  idiotic  or  feeble-minded  per- 
sons. 

Maudsley,  some  years  ago,  went  out  of  his  way  to  defend  the  rights  of 
moral  insanity.  "  It  maybe  witnessed  even  in  young  children,  who,  long  be- 
fore they  have  known  what  vice  meant,  have  evinced  an  entire  absence  of 
moral  feeling,  with  the  active  display  of  all  sorts  of  immoral  tendencies,  a 
genuine  moral  imbecility  or  insanity."  But  the  author  is  compelled  to  add 
that  "  associated  with  this  defect  there  is  frequently  more  or  less  intellectual 
deficiency,  but  not  always  ;  it  sometimes  happens  that  there  is  a  remarkably 
acute  intellect  with  no  trace  of  moral  feeling." 

There  are  some,  children  and  adults,  whose  intellect- 
ual faculties  are  on  a  far  higher  plane  than  their  moral 
qualities,  and  in  whom  the  latter  cannot  possibly  be  fostered. 
In  a  family,  one  of  five  or  six  children  may  be  the  only  one 
to  resist  the  influences  of  religious  and  secular  training. 
Many  authors  are  firmly  convinced  that  the  defect  in  mo- 
rality is  to  be  ascribed  primarily  to  an  intellectual  defect ; 
opposing  views  are  held  by  some  of  the  ablest  writers,  in- 
cluding Lombroso,  Maudsley,  and  Hack-Tuke.  Krafft- 
Ebing  urges  the  justice  of  retaining  moral  insanity  as  a 
clinical  form,  and  to  this  there  can  be  no  objections. 

Some  years  ago  I  saw  a  young  man  at  a  clinic  who  had  been  arrested  for 
assault  upon  his  mother,  whom  he  had  failed  to  kill.  He  was  entirely  indif- 
ferent to  the  charge  brought  against  him,  and  when  asked  whether  he 
thought  it  was  proper  to  kill  one's  mother,  answered,  "  You  might  as  well 
kill  your  mother  as  anyone  else."  The  young  man  had  received  no  intellect- 
ual or  moral  training,  no  religious  instruction,  had  grown  up  among  the 
most  degenerate  classes,  and  had  never  received  the  most  ordinary  moral 
teachings.  Naturally  the  moral  sense  was  deficient.  Such  a  condition  is 
embraced  in  Mendel's  definition  of  moral  insanity  as  that  form  which  is  either 
congenital  or  acquired  in  the  earlier  years  of  life,  and  is  characterized  by  im- 
becility associated  with  a  morbid  tendency  to  immoral  actions.  Binswanger 
holds  very  correctly  that  a  number  of  mental  diseases  lead  to  "  moral  idiocy." 

The  prognosis  of  the  condition  of  moral  insanity  is  abso- 
lutely unfavorable  ;  nothing  can  be  accomplished  except  by 
placing  such  children  with  moral  obliquities  under  the  stern 
discipline  of  a  reformatory  or  an  asylum. 

Epileptic  Insanity.* — Mental  derangement  associated 

*  Hysterical  Insanity  has  been  alluded  to  in  the  chapter  on  Hysteria  (pp.  86-88). 
The  author  was  at  first  inclined  to  discuss  the  entire  disease  in  this  division  on  mental 


538  THE  NERVOUS  DISEASES   OF   CHILDREN. 

with  epilepsy  has  been  alluded  to  in  a  previous  chapter. 
(See  page  67.)  It  is  only  necessary  in  this  connection  to  re- 
call the  fact  that  a  maniacal  attack  may  take  the  place  of  an 
ordinary  epileptic  seizure,  and  that  such  an  attack  is  charac- 
terized by  unusual  suddenness  and  violence.  Any  very  sud- 
den development  of.  mania  in  a  child  may  be  regarded  as  a 
symptom  of  possible  epilepsy,  even  if  other  epileptic  signs 
are  entirely  wanting.  In  addition  to  these  "  psychic  equiv- 
alents "  of  an  epileptic  seizure,  epileptic  children  exhibit  a 
marked  tendency  to  idiocy  and  dementia.  I  have  known 
of  the  condition  of  double  consciousness  in  an  epileptic 
aged  eighteen,  but  I  have  not  seen  it  in  younger  subjects. 
The  more  pronounced  symptoms  of  epileptic  insanity  are 
encountered  in  persons  past  the  age  of  puberty,  but  Wilder- 
muth  insists  that  only  about  twenty  per  cent,  of  infantile 
epileptics  exhibit  anything  like  a  normal  mental  condition. 

A  general  psychic  degeneration  is  a  most  marked  feature  of  epileptic  sub- 
jects. The  disease  is  very  common  among  criminals,  as  are  other  degenerative 
neuroses.  Alcoholism  in  the  parent  is  a  powerful  etiological  factor.  (De- 
jerine  states  that  in  37.7  per  cent,  of  three  hundred  and  fifty  epileptics  the 
father  was  a  drunkard.)  Moreover,  the  epileptic  boy  is  unable  to  attend 
school  and  falls  an  easy  victim  to  bad  associates. 

Paretic  Dementia,  the  serious  psychosis  of  middle  life,  characterized  by  a 
progressive  dementia,  delusions  of  grandeur,  and  a  long  series  of  physical 
symptoms  (inequality  and  immobility  of  the  pupils,  disturbances  of  speech, 
tremor  of  the  face  and  hands),  is  so  rare  in  childhood  that  we  do  not  feel 
warranted  in  including  it  among  the  forms  of  insanity  occurring  in  childhood. 
The  youngest  paretic  I  have  seen  was  a  man,  aged  nineteen  years.  Spitzka 
observed  one  at  eighteen  years,  Trumbull  observed  the  disease  in  a  boy  of 
twelve  years,  who  had  an  attack  of  hemiplegia  at  the  age  of  ten  years.  The 
autopsy  revealed  the  changes  of  paralytic  dementia.  The  cases  thus  far  ob- 
served in  youthful  subjects  did  not  take  as  rapid  a  course  as  in  the  adult  type. 

The  author  would  make  no  mention  of  the  disease,  were  it  not  for  an  ex- 
perience he  had  some  years  since  with  a  young  man  who  in  the  course  of  a 
few  weeks  became  exceedingly  hilarious,  developed  the  delusion  that  he  was 
enormously  wealthy,  showed  distinct  tremor  of  speech  and  of  hands,  inequal- 
ity of  the  pupils,  and  became  extravagant  and  grandiloquent.  The  diagnosis 
of  paralytic  dementia  was  made  by  competent  neurologists ;  the  boy  was  sent 
to  an  asylum,  where  all  his  symptoms  disappeared  after  a  few  weeks.  He 
has  been  entirely  well  since. 

diseases,  but  hesitated  to  do  so  because  the  marked  psychic  forms  of  hysteria  are  not 
often  observed  in  children.  The  mental  changes  in  chorea  have  been  referred  to  on 
page  115. 


INSANITY.  539 

Masturbation  and  Insanity. — There  is  not  sufficient 
reason  to  erect  a  special  type  to  be  called  " Masturbatic  In- 
sanity," for  the  habit  is  present  in  many  different  forn 
insanity,  particularly  in  young  subjects.  But  the  presence 
of  this  etiological  or  complicating  condition  is  always  notice- 
able, and  leaves  a  distinct  impression  upon  the  development 
of  the  various  psychoses.  In  many  cases  of  acute  derange- 
ment in  the  young,  in  the  various  forms  of  dementia  prae- 
cox,  it  is  often  an  important  and  grave  factor.  The  author 
has  had  under  his  observation  young  masturbators  who  at 
the  ages  of  ten  and  twelve  years  displayed  the  first  effects 
of  masturbation,  and  at  the  ages  of  sixteen  to  twenty  years 
presented  the  typical  symptoms  of  hebephrenia  (mental  en- 
feeblement,  silliness,  depression,  general  restlessness,  and 
irritability),  and  gradually  developed  a  striking  dementia. 
There  is  such  a  marked  resemblance  between  various  forms 
of  insanity  due  to  masturbation  that  one  is  sorely  tempted  to 
constitute  them  a  special  group*  The  following  account 
is  typical  of  many  the  author  could  give  :  At  the  age  of 
twelve  or  fourteen  years,  if  not  earlier,  the  boy  either  in- 
stinctively or  through  the  force  of  bad  example  begins  the 
habit.  For  a  time  it  has  no  marked  effect  upon  him  ;  he 
continues  in  his  studies,  but  does  not  get  on  quite  as  well 
as  he  formerly  did.  For  this  he  finds  all  sorts  of  excuses. 
He  is  supposed  to  be,  and  often  is,  subject  to  severe  head- 
aches ;  is  tired  in  the  morning,  late  in  rising,  loses  his 
pleasure  in  out-of-door  sports.  If  detected  at  this  stage 
and  convinced  of  the  viciousness  of  his  habit  he  may  make 
a  determined  effort  to  stop;  sometimes  he  succeeds;  more 
often  he  fails.  From  this  period  on,  lack  of  concentration 
upon  any  mental  effort,  general  irritability,  disturbed  sleep, 
loss  of  flesh,  are  the  prominent  symptoms.  Morbid  con- 
ceptions are  often  troublesome,  and  at  times  also  lead  to 
delusions  of  persecution.  A  young  man  under  my  care 
who  would  gaze  for  hours  upon  a  clock  heard  it  say,  "  De- 
tec-tive,  De-tec-tive,"  and  imagined  himself  run  down  by 
them,  but  was  even  then  too  stupid  to  give  any  reason  for 
such  persecution.  The  patient  leads  an  aimless,  silly  exist- 
ence ;  will  pore  for  hours  over  books  without  reading  them  ; 
does  nothing  of  his  own  initiative,  but  if  forced  to  walk 


540  THE   NERVOUS  DISEASES   OF   CHILDREN. 

or  to  take  exercise  of  some  sort  does  everything  in  a  me- 
chanical fashion.  If  he  be  the  son  of  wealthy  parents,  the 
effort  is  made  to  distract  him  by  travel,  but  all  is  in  vain  ; 
he  takes  no  interest  in  anything  and  simply  watches  for 
the  opportunity  to  indulge  in  masturbation.  Complete 
idiocy  and  dementia  are  the  ultimate  result.  Reference 
has  been  made  to  boys,  but  girls  also  fall  victims  to  the 
habit,  and  the  symptoms  they  present  are  similar  to  those 
specified  before.  The  prognosis  of  all  forms  of  insanity  due 
to  masturbation  is  extremely  unfavorable.  I  have  succeeded 
in  checking  the  habit  in  relatively  few  cases.  Many  patients 
continue  on  their  evil  course  in  spite  of  all  precautions.  It 
is,  if  anything,  more  difficult  to  control  the  habit  in  girls 
than  in  boys.  If  the  person's  reason  and  sense  of  shame 
can  be  appealed  to  the  chances  of  curing  him  of  the  prac- 
tice are  best;  the  previous  mental  calibre  is  an  important 
factor ;  the  successful  cases  I  have  seen  have  been  in  col- 
lege boys  and  other  brighft  lads.  If  the  habit  has  been  ac- 
quired by  imitation  it  can  be  checked  more  readily  than  if 
it  is  the  result  of  an  innate  instinct,  and  the  sign  of  a  degen- 
erated mental  state.  Extreme  watchfulness  and  the  liberal 
use  of  the  actual  cautery  to  the  spine  and  even  to  the  geni- 
tals are  the  only  possible  means  of  effecting  a  cure. 

The  prognosis  of  insanity  in  children  has  been  discussed 
with  reference  to  the  several  types.  It  is  well  to  emphasize 
the  fact  once  more  that  mental  derangement  in  childhood 
is  recovered  from  more  frequently  than  is  the  case  with  the 
insanity  of  later  years. 

A  word  in  addition  to  what  has  been  said  on  the  treat- 
ment of  the  various  types  of  insanity.  The  doctrines  of 
heredity  and  of  degeneration  are  taking  a  firm  hold  of  the 
medical  mind.  Psychologically  they  may  be  true  enough, 
but  no  one  has  yet  proved  that  morbid  hereditary  influences 
cannot  be  overcome  or  counteracted.  If  a  child  has  an  un- 
fortunate hereditary  predisposition  to  disease,  give  it  every 
possible  advantage  which  children  without  any  hereditary 
taint  enjoy.  The  separation  of  children  from  hysterical, 
epileptic,  or  otherwise  degenerate  parents,  is  not  insisted  on 
often  enough. 


CHAPTER   XXX. 

IDIOCY   AND   IMBECILITY. 

By  idiocy  we  designate  a  permanent  and  complete  im- 
pairment of  all  the  intellectual  faculties.  Imbecility  and 
feeble- mindedness  denote  lesser  degrees  of  mental  defi- 
ciency. Both  these  terms  are  used  to  designate  mental  de- 
ficiency in  a  brain  not  yet  fully  developed,  whereas  the 
term  "dementia"  is  applied  to  those  states  in  which  all 
the  faculties  are  lost  after  they  have  been  normally  de- 
veloped. Such  dementia  may  occur  at  times  in  relatively 
young  persons. 

Idiocy  is  of  special  interest,  not  only  because  of  its  frequent  occurrence, 
but  also  because  it  demonstrates  an  extreme  psychic  condition  due,  in  many 
instances,  to  tangible  changes  in  the  brain.  In  this  respect  it  is  not  unlike 
general  paresis,  and  the  mental  conditions  included  under  these  two  terms 
are  practically  the  only  two  psychic  diseases  whose  morbid  substratum  is 
tolerably  well  known  at  the  present  time.  In  one,  the  highest  structures 
of  the  brain  have  become  disorganized  and  are  undergoing  dissolution  after 
having  attained  normal  development,  while  in  the  other  defective  growth  or 
involution  takes  place  before  a  full  development  has  been  reached. 

In  former  years  idiots  were  much  neglected  ;  they  were 
considered  an  unfortunate  class  for  whom  but  little  could 
be  done,  and  whose  actual  condition  was  scarcely  worth 
studying  ;  but  during  the  past  twenty-five  years  or  more 
an  entire  change  has  taken  place  and  it  is  fair  to  say  that 
no  class  of  patients  has  been  more  frequently  the  subject 
of  study  than  those  with  defective  cerebral  development. 

The  earliest  impetus  was  given  in  this  direction  by  the  French  alienists, 
chief  among  them  being  Esquirol ;  a  little  latter  the  elder  Seguin  called  at- 
tention to  the  possibilities  of  improvement  in  these  patients  and  to  the  proper 
methods  of  training ;  and  within  the  past  decade  or  two,  the  interest  in  the 
morbid  conditions  underlying  idiocy  has  led  to  numerous  able  contributions. 


542  THE   NERVOUS  DISEASES    OF   CHILDREN. 

Among  English  authors  the  names  of  Crichton  Browne,  West,  Maudsley,  and 
Langdon  Down,  of  Clouston,  Ireland,  and  Shuttleworth,  deserve  special  men- 
tion ;  in  France,  Bourneville  has  been  the  most  successful  student  of  idiocy  ;  in 
Germany,  the  works  of  Schule,  of  Krafft-Ebing,  of  Emminghaus,  and  above  all, 
of  Griesinger,  have  contributed  most  to  the  advance  of  our  special  knowledge  of 
this  subject,  and  in  this  country  idiocy  has  been  studied  carefully  by  Ray, 
Seguin,  Mills,  Kerlin,  Spitzka,  Hurd,  Brush,  Peterson,  Barr,  and  the  author. 

Classification. — The  discussion  of  idiocy  has  been  not 
unlike  that  of  insanity.  Each  author  has  felt  called  upon 
to  subdivide  the  subject  and  to  establish  a  rational  classi- 
fication. The  result  has  been  that  no  two  authors  have  en- 
tirely agreed.  One  has  attempted  to  give  a  classification 
based  upon  the  etiology  of  the  condition,  another  upon  the 
pathology.  Down  has  attempted  a  division  based  upon 
ethnic  standards,  the  Mongolian,  the  Malay,  the  Indian,  and 
the  Ethiopian  types. 

For  purposes  of  convenience  we  will  divide  the  subject 
as  follows  : 

I.  Hereditary  Idiocy.  J  w     '     s 

(  (b)  Developmental. 

(  After  brain  or  skull  injuries  (including  birth  idiocies). 

II.  Acquired  Idiocy..  <  After  convulsions. 

(  After  infectious  diseases. 

III.  Myxedematous  Idiocy. 

I.  (a).  Hereditary  Congenital  Idiocy  includes  those  condi- 
tions in  which  the  brain  at  birth  is  deficient,  although 
this  defect  need  not  be  apparent  for  some  weeks  or  even 
months  after  birth.  In  the  chapter  upon  Arrested  Cerebral 
Development  I  have  enumerated  some  of  the  anatomical 
conditions  with  which  idiocy  is  associated,  and  to  this  part 
of  the  subject  we  shall  recur  later  on.  These  congenital  de- 
ficiencies may  be  the  result  of  disease  during  the  intra- 
uterine period. 

Congenital  idiocy  is  a  common  occurrence  in  families 
with  marked  neurotic  taint.  Parents  who  suffer  from  some 
form  of  insanity,  from  hysteria,  epilepsy,  or  chorea  are  apt 
to  engender  idiots.  Among  other  predisposing  conditions 
alcoholism  and  syphilitic  infection  of  either  parent  are  by 
far  the   most  frequent.     The   importance  of  alcoholism  in 


IDIOCY  AND   IMBECILITY.  543 

particular  cannot  be  overrated.  The  alcoholic  habit-  ol 
the  father  at  the  time  of  procreation  are  surely  a  potent  fac- 
tor. Syphilis  of  the  parent  must  be  taken  into  account. 
Premature  delivery  is  not  uncommon  in  syphilitic  cases, 
and  an  imperfectly  developed  brain  at  the  time  of  birth 
may  be  the  cause  of  idiocy. 

Intermarriage  is  supposed  by  many  to  be  a  direct 
cause  of  idiocy  in  children,  and  by  others  the  influence  of 
such  marriages  is  absolutely  denied.  The  truth  lies  mid- 
way. In  families  without  a  hereditary  taint  intermarriages 
may  be  quite  harmless,  but  if  there  is  any,  and  even  the 
slightest  taint  of  insanity,  or  oi  any  other  serious  nervous 
affection,  that  taint  becomes  intensified  by  such  an  union ; 
and  since  few  families  are  entirely  free  from  every  such 
tendency  the  rule  holds  good  that  in  the  majority  of  cases 
intermarriages  are  harmful. 

Traumatism  during  pregnancy  is  another  factor  in  the 
development  of  idiocy  in  the  child.  As  the  influences 
brought  to  bear  are  prenatal  in  origin,  these  cases  may  be 
included  under  the  heading  of  hereditary  idiocy  ;  and  there 
is  all  the  more  reason  to  do  this  as  such  injuries  to  the 
mother  are  more  likely  to  be  harmful  to  the  normal  devel- 
opment of  a  child's  brain  in  families  with  neurotic  taint  than 
in  families  whose  history  is  entirely  negative. 

I.  (b)  Developmental  Idiocy  includes  that  class  of  cases  in 
which  the  idiocy  becomes  apparent  at  certain  well-marked 
periods  of  life — during  dentition,  for  instance,  and  at  pu- 
berty. 

II.  Acquired  Idiocy. — This  class  includes  a  very  large 
number  of  conditions,  due  to  the  most  varying  accidents 
and  diseases.  First  and  foremost,  although  the  cause  is 
operative  at  the  time  of  birth,  we  must  include  that  large 
number  of  fdiocies  due  to  traumatism  during  labor.  The 
same  conditions  which  give  rise  to  cerebral  birth  palsies 
(prolonged  labor,  instrumental  delivery)  also  give  rise  to 
birth  idiocy.  Other  things  being  equal  the  application  of 
instruments  is  not  nearly  as  harmful  as  excessively  pro- 
longed labor.  In  the  histories  of  children  who  have  be- 
come idiotic,  it  is  often  stated  that  the  child  was  asphyxi- 
ated at  birth  ;  that  it  had  frequent  spasms  during  the  earlier 


544  THE  NERVOUS  DISEASES   OF   CHILDREN. 

weeks  of  life,  and  that  it  exhibited  marked  rigidities  and 
palsies  of  the  extremities. 

According  to  Mitchell,  in  57  of  494  cases  of  idiocy  labor  lasted  for  more 
than  thirty-six  hours  ;  4  of  them  were  born  with  unusual  haste  ;  in  22  cases 
forceps  were  applied,  and  9  of  these  showed  the  impressions  of  the  for- 
ceps on  the  head  after  birth ;  4  of  them  were  born  by  version  ;  in  6  cases 
there  was  breech  presentation  ;  1 1  were  twins,  and  9  were  born  prematurely  ; 
29  were  born  asphyxiated  and  supposed  to  be  dead  ;  89  were  the  last  children 
of  their  respective  mothers. 

Birth  idiocy  is  allied  to  another  form  due  to  injuries  to 
the  head  later  in  life  ;  a  fall  from  a  bench,  from  a  chair, 
from  a  cradle,  is  often  sufficient  to  cause  serious  disturbance, 
more  particularly  in  those  children  who  are  predisposed 
by  inheritance  to  mental  disease.  It  is  possible  that  the 
easier  rupture  of  the  blood-vessels  in  those  who  have  in- 
herited a  syphilitic  or  alcoholic  taint  may  account  for  the 
fact  that  while  some  children  escape  injury  after  severe 
falls  others  are  seriously  affected  by  relatively  slight  acci- 
dents. 

Among  the  causes  of  acquired  idiocy  none  is  more  im- 
portant to  my  mind  than  convulsions.  The  entire  devel- 
opment of  a  child  may  be  normal  until  a  convulsion  occurs, 
whether  as  a  result  of  some  intestinal  derangement  or  as 
the  precursor  of  an  acute  infectious  disease.  From  this 
time  onward  mental  decadence  sets  in,  and  a  child  that 
was  previously  healthy  and  of  normal  mental  development 
begins  to  exhibit  more  and  more  marked  mental  defects 
until  it  reaches  the  condition  of  complete  idiocy,  in  which  it 
may  remain  for  many  years.  There  is  no  difficulty  in  ex- 
plaining this  occurrence,  for  the  tremendous  venous  stasis 
that  occurs  during  the  acme  of  a  convulsion  is  sufficient  to 
cause  rupture  of  the  pial  blood-vessels,  and  a  very  consider- 
able hemorrhage  may  follow  ;  meningo-encephalitis,  termi- 
nating in  a  general  sclerosis,  is  the  natural  result  and  the 
direct  cause  of  the  mental  defect.  The  acute  infectious 
diseases  are  often  responsible  for  the  development  of  idiocy. 
In  what  manner  this  cerebral  change  is  brought  about  is 
not  easy  to  state,  but  I  believe  that  the  cases  thus  produced 
are  relatively  few,  and  that  of  these  some  are  due  to  an  in- 


IDIOCY  AND  IMBECILITY. 


545 


fectious  encephalitis,  and  others  to  the  convulsions  accom- 
panying these  acute  infectious  diseases. 

Idiocy  is  developed  not  infrequently  after  acute  menin- 
gitis early  in  childhood.  Idiocy,  blindness,  deaf-mutism — 
all  these  conditions  separately,  and  sometimes  conjointly, 
are  the  unfortunate  results  of  early  meningeal  disease.  The 
explanation  of  this  condition  is  relatively  simple,  for  the 
meningitis  and  the  meningo-encephalitis,  if  they  last  long 


Fig.  135. — Hydrocephalic  Idiot.     (Peterson.) 


enough,  may  lead  to  a  general  sclerosis  and  atrophy  of  the 
cortical  substance,  which  will  prevent  the  further  normal 
development  of  the  brain. 

The  association  of  idiocy  with  hydrocephalus  is  com- 
mon, but  as  this  condition  is  generally  a  secondary  state, 
both  the  idiocy  and  the  hydrocephalus  are  the  result  of  the 
primary  disease.  (See  Fig.  I35-)  With  the  gradual  increase 
of  the  hydrocephalic  fluid,  however,  the  cortical  function 
naturally  becomes  impaired  ;  there  are  therefore  few  chil- 
dren with  unusually  large  heads  whose  mental  condition  is 
at  all  normal,  though  it  is  quite  remarkable  to  what  extent 
the  hydrocephalus  may  increase  before  an  absolute  aboli- 


546  THE  NERVOUS  DISEASES   OF   CHILDREN. 

tion  of  function  takes  place.  This  is  true  not  only  of  the 
general  mental  condition,  but  also  of  the  special  sensory 
functions  of  the  cortex. 

Idiocy  and  epilepsy  are  intimately  associated  with  one 
another.  In  all  cases  in  which  epilepsy  has  been  developed 
early  in  life,  or  in  which  the  epileptic  seizures  are  frequent, 
there  is  a  natural  tendency  to  mental  deterioration.  In  the 
adult  we  speak  of  epileptic  dementia,  in  younger  persons  of 
epileptic  idiocy.  There  are,  no  doubt,  gross  changes  in  the 
brains  of  such  subjects,  and  in  many  the  seeds  of  both  the 
epilepsy  and  the  idiocy  may  be  traced  to  injury  either  at 
birth  or  during  the  first  years  of  life.  Thus  not  only  the 
idiocy  but  the  epilepsy,  as  well  as  the  palsy,  may  be  the  re- 
sult of  meningeal  hemorrhage  occurring  during  labor  or  in 
very  early  childhood. 

Symptoms. — The  chief  characteristic  of  idiocy  is  the 
lack  of  ordinary  mental  conceptions.  The  brain  is  not  able 
to  receive  impressions  from  the  outer  world,  and  if  such 
are  received,  it  is  not  able  to  utilize  them  in  anything 
like  normal  fashion,  nor  to  form  concepts  or  judgments. 
In  some  instances  the  brain  represents  an  entire  blank,  in 
others  a  few  impressions  have  been  received,  and  these 
have  been  developed  into  imperfect  concepts.  This  abso- 
lute mental  deficiency  is  present  in  typical  idiots,  but  there 
are  varying  degrees  of  mental  deficiency  or  of  mental  devel- 
opment in  imbeciles  or  feeble-minded  persons. 

It  is  scarcely  necessary  to  cite  cases  in  order  to  establish 
the  clinical  features  of  complete  idiocy,  for  they  are  too  well 
known  even  to  laymen  to  need  elucidation. 

If  idiocy  is  complete  the  child  or  the  adult  may  not  be 
able  to  recognize  its  own  parent.  He  fails  to  recognize  any 
object  or  the  use  of  such,  and  of  course  is  not  able  to  under- 
stand or  appreciate  what  is  said  to  him.  In  extreme  in- 
stances the  idiot  is  practically  nothing  more  than  a  highly 
organized  vegetating  organism,  truly  animal-like  in  all  his 
actions  without  the  slightest  trace  of  human  intellect. 
Through  faulty  development  of  speech  the  defect  in  the 
general  mental  condition  of  the  child  first  becomes  notice- 
able. If  there  is  a  lesser  degree  of  idiocy,  the  child  may 
master   a   few    concepts,    may    understand    a   few    uttered 


IDIOCY  AND  IMBECILITY.  547 

sounds,  or  may  be  able  to  pronounce  such  simple  words 
as  mamma,  papa,  and  the  like.  He  may  recognize  the 
use  of  objects,  may  be  capable  of  slight  training  and  in- 
struction, so  that  the  ideas  of  cleanliness,  and  of  mine  and 
thine  may  be  impressed  upon  his  mind. 

From  these  rather  marked  forms  of  idiocy  and  mental 
imbecility  there  is  every  possible  gradation  until  we  reach 
those  types  in  which  speech  is  only  a  little  deficient,  but 
the  mental  horizon  is  evidently  narrowed  down  so  that  rel- 
atively few  concepts  are  formed,  abstract  ideas  are  almost 
entirely  wanting,  and  the  imperfect  mental  status  is  deter- 
mined by  the  lack  of  ordinary  motives  for  action,  by  the 
awkwardness  in  intercourse  with  other  people,  and  above 
all  by  the  inability  of  the  individual  to  cope  with  others  in 
the  struggle  for  existence.  The  inferiority  of  the  individ- 
ual is  discovered  not  in  the  family  circle  in  which  all  con- 
ditions are  favorable,  but  when  the  child  is  thrown  into 
competition  with  others  at  school.  If  the  boy  or  young 
man  is  compelled  to  seek  a  living  for  himself,  the  feeble- 
minded youth  is  pushed  to  the  wall  under  such  condi- 
tions. That  such  persons,  moreover,  cannot  appreciate  the 
reason  for  their  failure  to  make  a  success  of  life  goes  with- 
out saying.  That  they  are  rarely  capable  of  developing 
the  higher  religious  and  moral  ideas  is  equally  true.  The 
criminal  classes  are  recruited  largely  from  the  category  of 
feeble-minded  persons  in  whom  the  ordinary  ideas  of  right 
and  wrong  have  not  been  engendered  by  early  training. 

The  general  deficiency  in  intellect  is  supposed  to  be  offset  occasionally  by 
a  peculiar  development  of  the  mind  in  some  one  direction.  Stories  to  this 
effect  have  been  common,  and  have  been  repeated  by  one  author  after  the 
other  ;  Griesinger's  case  has  become  rather  famous  of  an  idiot  who  was  said 
to  have  constructed  a  perfect  model  of  a  man-of-war  without  having  had 
any  conception  of  geometrical  designs.  Many  idiots  are  said  to  possess  spe- 
cial talent  in  the  use  of  cards.  Drobisch  described  an  idiotic  boy,  who  after 
reading  over  a  single  page  was  able  to  repeat  word  for  word,  even  if  it  was 
in  Latin,  which  he  did  not  at  all  understand.  I  have  had  under  observation  a 
young  imbecile,  a  boy  aged  fourteen,  who  takes  special  delight  in  reading  and 
remembering  each  sign  that  he  passes  on  the  streets,  and  he  can  without 
difficulty  repeat  fully  two  hundred  names  in  the  order  in  which  they  occur 
on  the  avenues.  He  has  a  true  passion  for  signs,  and  as  he  passes  along  the 
streets  his  attention  is  riveted  upon  nothing  else.     Some  of  the  most  astound- 


54§  THE  NERVOUS  DISEASES   OF   CHILDREN. 

ing  lightning  calculators  have  been  weak-minded,  if  not  imbecile,  in  everything 
else  excepting  memory  of  figures.  It  is  not  fair,  however,  to  turn  this  about 
and  to  claim  that  every  lightning  calculator  must  necessarily  be  an  imbecile, 
for  there  is  no  doubt  that  some  normal  minds  can,  by  special  training,  reach 
an  inordinate  development  in  some  one  particular  direction. 

As  we  are  interested  chiefly  in  the  mental  condition  of 
children,  we  must  devote  a  little  more  time  to  the  early  rec- 
ognition of  idiocy.  In  cases  of  very  marked  mental  defi- 
ciency the  disordered  condition  of  mind  can  be  discovered 
in  the  first  months  of  life.  In  fact  if  parents  and  physicians 
had  a  proper  understanding  of  such  conditions,  the  mental 
defect  could  be  discovered  very  much  earlier  than  it  gen- 
erally is.  It  is  most  instructive  in  this  connection  to  con- 
sider the  observations  of  Preyer,  who,  in  his  famous  treatise 
on  the  psychic  development  of  the  child,  shows  how  early 
the  mental  processes  of  a  healthy  child  can  be  discerned. 
It  is  a  surprise  to  most  of  us  to  learn  from  him  that  a  child 
in  the  second  day  of  life  was  able  to  distinguish  between 
light  and  darkness  ;  that  at  a  very  early  day  it  appreciates 
the  sounds  of  words,  and  that  in  its  cooing  utterances  it 
exhibits  evidence  of  considerable  cerebration.  Very  few 
people  will,  of  course,  observe  children  as  Preyer  observed 
his  own  child,  but  mental  deficiency  can,  as  a  rule,  be  made 
out  if  the  parent  or  the  physician  take  the  trouble  to  com- 
pare the  actions  of  a  child  with  the  average  healthy  child 
at  the  same  age.  It  is  important,  therefore,  to  note  the  time 
of  life  at  which  children  may  be  expected  to  exhibit  certain 
signs  of  intelligence.  (See  page  7.)  If  children  have  not 
acquired  speech  at  the  end  of  two  and  a  half  years,  and  par- 
ticularly if  they  fail  to  understand  what  is  spoken,  it  is  fair 
to  infer  that  there  will  be  a  distinct  mental  defect  later  on. 

Wildermuth  has  taken  the  trouble  to  tabulate  a  number  of  the  more  im- 
portant stigmata  of  degeneration  commonly  found  in  idiots.  Eighty-two  per 
cent,  of  all  idiots  according  to  these  statistics  present  some  such  signs.  There 
were  abnormal  conditions  of  the  fundus  in  6  of  142  cases ;  malformations  of 
the  external  ear  in  53  ;  abnormal  position  of  the  teeth  in  32  ;  flattening  of 
the  hard  palate  in  1 1  ;  highly  arched  palate  in  30  ;  prognathism  in  9 ;  ex- 
cessive thickening  of  the  skin  in  9 ;  asymmetry  of  the  face  in  25  ;  and  abnor- 
malities of  the  genital  organs  in  8  cases.  Among  the  functional  stigmata 
Wildermuth  refers  to  anomalies  of  the  tendon  reflexes  in  sixty  per  cent. ; 
inco-ordination  of  the  lower  extremities  in  ten  per  cent. ;  and  in  six  per  cent. 


IDIOCY  AND  IMBECILITY. 


549 


squinting  occurred.  It  is  a  curious  fact  that  these  signs  of  degeneration  are 
present  not  only  in  the  truly  congenital  forms  of  idiocy,  but  also  in  those 
which  have  been  acquired  some  time  after  birth. 

The  mere  appearance  of  the  child  or  of  the  youth  will 
often  be  sufficient  to  lead  to  the  suspicion  of  idiocy  without 
an  examination  of  the 
mental  condition  itself. 
The  entire  absence  of 
speech,  or  of  defective 
speech  in  many  cases,  will 
point  the  same  way.  Cau- 
tion should,  however,  be 
exercised  in  not  mistaking 
deaf- mutism  for  idiocy, 
and  if  a  child  cannot 
speak,  careful  examina- 
tion should  be  made  in 
other  ways  to  determine 
its  intelligence  ;  not  rare- 
ly, however,  deaf-mutism 
and  idiocy  are  combined, 
and  at  times  the  former 
has  been  the  direct  or  in- 
direct cause  of  the  latter. 

Among  idiots  the  de- 
fects of  skull  formation 
are  of  especial  interest. 
The  microcephalic  skull 
is  particularly  frequent, 
and  is  either  small  in  all 
its  dimensions  or  ample 
in  some  and  very  defi- 
cient in  others.  (See  Fig. 
136.)  The  transverse  and 
occipital  diameters  may  be  entirely  normal,  indeed  the 
horizontal  circumference  may  be  up  to  the  average,  and 
yet  if  some  such  heads  are  examined  it  will  be  found  that 
the  frontal  portion  is  unusually  small,  possibly  receding, 
while  the  middle  and  occipital  portions  may  be  entirely 
normal.     Such  discrepancies  and  such  asymmetry  should 


Fig.  136. — Congenital  Idiot ;  Microcephalic 
Skull ;  Extreme  Contractures  of  Adductor 
Muscles  of  Thighs.  (From  a  photograph 
kindly  furnished  by  Dr.  Peterson.) 


550  THE   NERVOUS  DISEASES   OF   CHILDREN. 

be  carefully  noted,  for  it  is  more  important  than  slight 
deficiencies  in  the  total  measurements.  Smallness  of  the 
anterior  half  of  the  skull  with  receding  forehead  proves 
either  that  there  is  very  little  room  for  the  brain,  or  that 
the  brain  being  small  requires  very  little  space.  Asymmetry 
of  the  skull  is  particularly  frequent,  as  has  been  shown  by 
Fisher  and  Peterson,  in  those  cases  in  which  idiocy  is  asso- 
ciated with  early  infantile  hemiplegia.  Another  point  to  be 
remembered  is  that  irregularities  in  the  structure  of  the 
skull  are  not  uncommon  in  healthy  individuals,  and  that  as 
long  as  the  cubic  contents  of  the  cavity  of  the  skull  are 
near  the  average,  defects  of  one  kind  or  the  other  appear  to 
play  a  very  small  part.  In  various  parts  of  this  book  I 
have  referred  to  my  belief  that  the  growth  of  the  skull  is 
dependent  largely  upon  the  condition  of  the  brain  within, 
and  this  accounts  for  the  fact  that  in  idiocy  acquired  after 
convulsions  the  growth  of  the  skull  ceases  with  the  dis- 
turbed condition  of  the  brain  ;  microcephalus  is  found 
among  acquired  idiots  as  well  as  among  congenital  idiots. 

The  general  restlessness  of  idiots  is  characteristic.  In 
my  lecture-room  I  allowed  these  children  full  sway  in  order 
to  demonstrate  this  special  feature  to  my  class.  They  are 
about  the  only  patients  who  concern  themselves  little  about 
the  presence  of  the  students,  and  who  roam  about  the  entire 
room  in  an  uneasy  fashion,  taking  hold  of  everything,  pul- 
ling down  what  they  can,  and  always  fearless  of  the  conse- 
quences. If  they  cannot  employ  themselves  in  any  other 
way,  they  will  keep  up  a  constant  motion  with  the  fingers, 
twisting  and  braiding  them,  allowing  the  nails  to  scrape  the 
skin  off  the  fingers.  They  bite  their  nails  and  are  apt  to 
tear  their  garments.  There  is  frequently  dribbling  and 
drooling,  and  if  opportunity  is  offered,  these  children  are 
particularly  fond  of  putting  their  tongues  to  the  window- 
panes,  or  any  other  cold  object.  The  general  awkward- 
ness of  the  movements,  excitability  and  irritability  of  tem- 
per, together  with  a  peculiarity  of  carriage  and  blankness 
of  facial  expression,  complete  the  clinical  picture  of  idiocy 
and  imbecility. 

Imbeciles  of  lesser  degree  often  escape  detection  ;  some  of  them  are  suffi- 
ciently conscious  of  their  defects  to  conceal  them  in  the  presence  of  others. 


IDIOCY  AND   IMBECILITY.  5  5  j 

As  they  grow  older  the  deficiency  in  the  mental  and  moral  make-up  is  evi- 
denced in  the  excessive  development  of  the  animal  appetites.  Masturbation 
is  developed  in  early  years,  the  effect  of  which  adds  to  the  mental  deteriora- 
tion. If  the  sexual  appetite  is  thoroughly  aroused,  gratification  is  sought  in 
the  most  outrageous  fashion,  and  intercourse  may  be  attempted  with  children 
or  old  women.  Giraud  referred  to  an  idiot  who  attempted  to  rape  his  own 
sister.  All  the  ordinary  feelings  of  shame  and  modesty  are  wanting.  In  some 
instances  the  defect  is  a  little  more  marked  in  the  emotional  or  moral  spheres 
than  in  the  intellectual.  Such  a  condition  has  generally  been  termed  one  of 
moral  imbecility  ;  but  it  is  always  associated  with  a  decided  intellectual  defect. 
Weak-minded  and  imbecile  children  commit  crimes  either  from  defective 
judgment  as  to  the  consequences  or  from  the  simple  desire  to  gratify  their 
passions  and  impulses.  The  desire  to  witness  a  grand  spectacle  or  to 
revenge  himself  upon  others,  has  led  many  an  imbecile  to  set  fire  to  houses, 
regardless  of  all  consequences. 

Pathology. — We  have  stated  that  idiocy  is  rarely  a 
primary  condition,  that  it  is  frequently  associated  with 
other  conditions  which  point  to  serious  brain  trouble.  A 
complete  account  of  the  pathology  of  idiocy  would  include 
the  terminal  stage  of  very  many  different  brain  diseases. 
While  it  is  interesting  to  note  these  various  terminal  states 
it  helps  us  but  little  in  determining  the  primary  lesion. 
Thus  Wilmarth  has  described  one  hundred  brains  of  idiots. 
Among  these  were  found  conditions  of  sclerosis  with  atro- 
phy, of  tuberous  sclerosis,  of  general  diffuse  sclerosis.  He 
described,  furthermore,  brains  in  which  the  most  marked 
features  were  degenerative  changes  in  the  vessels  and  higher 
nerve-cells.  He  also  mentioned  hydrocephalus,  general  atro- 
phy, and  the  like,  so  that  it  would  be  difficult  from  this 
enumeration  to  make  any  sort  of  inference  as  to  the  primary 
morbid  state.  The  attempt  should  be  made  to  distinguish 
between  the  primary  and  terminal  states,  and  thus  help  us 
to  push  on,  however  little,  toward  a  final  solution  of  the 
question.  I  believe  we  shall  do  best  if  we  accept  the  clini- 
cal division  of  idiocy  adopted  in  this  chapter,  and  endeavor 
to  arrange  the  known  morbid  processes  as  far  as  possible 
under  these  larger  clinical  subdivisions. 
41 


552 


THE  NERVOUS  DISEASES   OF   CHILDREN. 


Hereditary  idiocy. 


Acquired  idiocy. 
(a)  Birth  palsies. 


(b)  After  acute  dis- 
eases and  convul- 
sions. 

(c)  From  trauma- 
tism. 


Primary  Lesions. 


Large  defects  ;  hemicephalus 
or  entire  absence  of  a  con- 
siderable portion  of  a  hemi- 
sphere. 

Partial  defects  (porenceph- 
aly) ;  most  frequently  in 
motor  regions.  Single  or 
double. 

Small  brain  (microcephalus)  ; 
all  parts  equally  developed, 
or  arrest  of  development 
more  marked  in  frontal  and 
occipital  portions  than  in 
other  parts. 

Agenesis  corticalis ;  brain  ap- 
parently normal  or  showing 
only  slight  changes  in  ex- 
ternal configuration  ;  mal- 
development  and  disintegra- 
tion of  cells  and  fibres  of 
cortex. 

Intra-uterine,  inflammatory,  or 
vascular  disturbances,  such 
as  encephalitis,  thrombosis, 
and  hemorrhage. 

Meningeal  hemorrhage. 


Meningitis,  encephalitis  (sim- 
ple or  hemorrhagic)  ;  throm- 
bosis (specific  endarteritis). 

Meningeal  hemorrhage ;  en- 
cephalitis. 


Terminal  States. 


Same  ;  often  compensatory  de- 
velopment of  hydrocephalus 
with  large  cysts. 

Same  ;  with  addition  of  hydro- 
cephalus, local  cysts,  and  gen- 
eral sclerosis  with  atrophy. 

Small  brain,  often  hard  and 
sclerotic,  sometimes  compen- 
satory hydrocephalus. 


Same  ;  no  sign  of  inflamma- 
tory conditions,  blood-vessels 
scanty  ;  some  external  hydro- 
cephalus. 


Meningo  -  encephalitis  ;  sclero- 
sis, localized  or  diffuse  ;  gen- 
eral atrophy  ;  cysts. 


Meningo  -  encephalitis,  local 
cysts,  sclerosis,  and  general 
atrophy. 

Meningo- encephalitis,  sclero- 
sis, diffuse  atrophy. 

Meningoencephalitis,  abscess, 
sclerosis,  and  atrophy. 


That  there  are  other  conditions  which  occasionally  lead 
to  idiocy  cannot  be  doubted,  but  they  are,  I  am  sure,  not 
nearly  as  important  nor  as  frequent  as  those  mentioned  in 
the  above  table.  If  hereditary  syphilis  is  the  etiological 
factor  it  is  more  than  likely  to  give  rise  to  idiocy  through 
the  medium  of  local  meningitis,  encephalitis,  or  of  throm- 
bosis with  its  subsequent  changes.  Mills  insists  on  idiocy 
of  toxic  origin,  and  includes  under  this  term  cases  due  to 
acute  poisoning,  or  those  following  acute  infectious  dis- 
eases, such  as  measles,  scarlet  fever,  and  the  like. 

Diagnosis. — Idiocy  is  recognized  easily  enough,  except 
in  very  young  children  ;  a  comparison  with  the  attainments 
of  other  children  of  the  same  age  will  help  to  establish  the 
fact  of  an  inferior  mental  development.  If  a  child  presents, 
in  addition  to  the  mental  symptoms,  any  of  the  physical 
stigmata  of  degeneration,  the  probability  of  its  remaining 


IDIOCY  AND  IMBECILITY.  553 

an  idiot  is  very  great.  Defective  development  of  speech 
after  a  child  has  passed  the  third  year  is  also  an  important 
aid  in  diagnosis,  if  this  condition  is  not  the  result  of  deaf- 
mutism. 

The  Prognosis  in  the  majority  of  cases  of  idiocy  is  bad. 
If  a  child  has  not  learned  to  speak,  and  has  not  acquired 
the  simplest  concepts  at  the  age  of  three  or  four  years  it  will 
remain  backward  for  all  time.  In  the  case  of  imbeciles  of 
lesser  degree  the  prospect  is  not  quite  so  gloomy.  In  this 
condition  more  can  be  expected  from  the  careful  training 
on  the  part  of  parents  and  teachers. 

Treatment. — The  treatment  of  idiots  is,  as  a  rule,  re- 
stricted to  watching  over  their  physical  development  and 
securing  for  them  proper  hygienic  surroundings.  In  fam- 
ilies such  children  are  a  torment  to  their  parents  and  a  bad 
example  to  brothers  and  sisters.  Their  separation  from 
home  is  advisable,  and  should  be  urged  in  spite  of  all  senti- 
ment to  the  contrary.  The  patients  enjoy  the  greater 
liberty  which  special  institutions  afford  them,  and  the  family 
is  better  off  without  them. 

Imbeciles  and  weak-minded  children  require  more  care- 
ful consideration.  By  the  most  diligent  training  at  the 
hands  of  experienced  persons  much  can  be  done  for  them 
to  bring  their  behavior  and  their  physical  condition  as 
closely  as  possible  to  that  of  the  average  child.  I  am  a 
thorough  believer  in  pedagogic  methods  and  of  such  only. 
If  at  all  possible,  such  children  should  be  intrusted  at  a  very 
early  day  to  a  competent  teacher,  who  will  take  the  time 
to  study  the  peculiarities  of  the  individual,  and  who  will 
fasten  upon  and  endeavor  to  develop  the  few  signs  of  an 
intellectual  awakening.  It  is  often  surprising  to  see  how 
much  can  be  accomplished  with  these  unfortunates.  In 
some  instances,  however,  the  task  is  a  hopeless  one,  and  all 
the  evidences  of  imbecility  can  rarely  be  eradicated.  By 
careful  calisthenic  exercises,  and  proper  gymnastic  training, 
much  can  be  done  to  avoid  the  outward  appearance  of  im- 
becility to  which  parents  so  seriously  object.  If  special  at- 
tention is  paid  to  the  development  of  speech,  defects  in  this 
direction  can  be  corrected.  The  larger  institutions,  such  as 
the  Bicetre  in  Paris,  and  the  various  institutions  for  the 


554  THE  NERVOUS  DISEASES   OF   CHILDREN. 

feeble-minded  in  this  country,  attain  a  fair  measure  of  suc- 
cess. But,  after  all,  only  those  who  possess  a  modicum  of 
intellectual  development  can  be  benefited  by  these  meth- 
ods, whereas  the  severer  grades  of  imbeciles  and  of  idiots 
cannot  be  improved  by  them. 

Cranial  surgery  has  been  looked  to  as  a  final  resort.  If 
the  imbecility  or  the  idiocy  is  distinctly  due  to  a  small  skull, 
and  this  small  skull  is  the  result  of  a  premature  synostosis 
of  the  sutures,  the  surgical  procedures,  as  practised  by  Lan- 
nelongue,  Keen,  Gerster,  and  others,  may  be  allowable  ;  but, 
after  all  that  can  be  said  on  this  subject  has  been  heard, 
there  is  but  little  reason  to  expect  much  improvement. 
The  slight  changes  that  have  been  noticed  and  have  been 
reported  in  children  after  craniectomy  scarcely  warrant 
the  procedure,  and,  as  has  been  intimated  in  a  previous  sec- 
tion^ the  dangers  of  the  operation  are  so  great  that  crani- 
ectomy should  only  be  practised  at  the  urgent  request  of 
the  parents,  and  after  ever)7  other  method  has  been  given  a 
fair  trial  and  has  failed.  In  those  patients  in  whom  there  is 
evidence  of  a  defective  cerebral  development,  independently 
of  the  condition  of  the  skull,  any  operation  is,  on  a  priori 
grounds,  entirely  useless. 

Medication  by  drugs  is  rarely  beneficial ;  yet  some  phy- 
sicians will  undoubtedly  be  impelled  by  the  evidence  of  an 
existing  anaemia,  of  rickets,  or  of  hereditar}^  s}rphilis,  to  ex- 
hibit the  drugs  commonly  used  in  these  conditions. 

III.    MYXCEDEMATOUS   IDIOCY — SPORADIC    CRETINISM. 

This  condition,  which  was  well  described  by  Bourneville  many  years  ago, 
deserves  special  mention.  The  mental  condition  is  a  very  striking  feature  of 
these  children,  but  it  is  simply  a  part  of  the  general  stunting  of  the  physical 
and  mental  growth.  The  condition  itself,  although  a  very  rare  one,  is  of 
more  interest  at  the  present  day  in  connection  with  the  satisfactory  studies 
that  have  been  made  regarding  the  cause  and  the  treatment  of  myxcedema.  I 
have  associated  myxcedematous  idiocy  with  sporadic  cretinism,  because  the 
two  seemed  to  me  to  be  very  closely  related,  the  cretin  representing,  how- 
ever, a  more  intense  development  of  the  affection  than  is  met  with  in  those 
cases  described  as  myxcedematous  idiocy  by  Bourneville.  Cases  of  sporadic 
cretinism  in  every  way  resembling  the  endemic  cretinoid  cases,  so  common 
in  the  mountainous  districts  of  Europe,  occur  also  in  a  few  American  re- 
gions, as  in  the  mountains  of  Vermont  and  California.     The  cases  which  we 


IDIOCY  AND  IMBECILITY. 


555 


have  the  opportunity  to  see  in  the  larger  American  cities  are  from  the  for- 
eign element  of  the  population,  and  though  we  speak  of  them  as  cases  of 
sporadic  cretinism,  they  occur  generally  in  descendants  of  families  who  have 
lived  in  regions  in  which  cretinism  has  been  endemic. 

The  child,  whose  picture  is  reproduced  in  this  chapter,  is  a  typical  case  of 
this  kind.  The  boy  was  born  of  healthy  Prussian  parents,  who  immigrated 
to  this  country  some  seven  or  eight  years  ago.  He  has  always  been  healthy, 
but  ever  since  he  was  a  year  and 
a  half  of  age,  the  parents  have 
been  struck  by  the  fact  that 
he  did  not  seem  to  continue 
growing  as  the  other  children 
in  the  family  did.  This  stunt- 
ed physical  growth  was  asso- 
ciated with  an  impairment  in 
the  development  of  speech  and 
of  all  other  faculties. 

The  child  might  not  unnat- 
urally* at  the  age  of  twelve,  be 
taken  to  be  three,  or  not  much 
more  than  three  years  old.  The 
boy  has  learned  to  speak  a  few 
words,  is  able  to  call  his  par- 
ents and  some  near  relatives. 
His  affections  are  well  devel- 
oped, as  was  evidenced  when 
he  was  separated  from  his  father 
and  placed  in  the  hospital,  and 
he  shows  considerable  aptitude 
in  playing  with  other  children, 
but  not  quite  as  much  as  a 
healthy  child  of  three  years  of 
age  would.  As  for  his  mental 
condition,  it  is  not  equal  to  that 
of  a  child  of  three  years;  be- 
yond the  expression  of  his  sim- 
ple wants,  he  exhibits  remarka- 
bly few  signs  of  mentality. 

The  disease  is  characterized 
by  a  peculiar  glossy  appearance  of  the  skin,  prominent  lips,  receding  fore- 
head, and  a  peculiar,  stubbed  nose.  Compared  with  the  rest  of  the  body  the 
stomach  is  inordinately  large ;  the  tongue  is  relatively  thick,  and  very  often 
protrudes  from  the  mouth.  All  children  with  myxcedematous  disease  so 
closely  resemble  each  other  that  they  might  be  supposed  to  belong  to  one  and 
the  same  family.  The  examination  of  the  neck  fails  to  reveal  any  trace  of 
the  thyroid  gland. 

Etiology. — The  etiology  of  myxcedematous  idiocy,  as  well  as  of  spora- 


FiG.  137. — Case  of  Myxoedema  with  Idiocy.    Pa- 
tient Twelve  Years  Old  ;  Dwarfish  in  Stature. 


55^  THE  NERVOUS  DISEASES   OF  CHILDREN. 

die  cretinism,  is  practically  unknown,  except  that  it  is  very  apt  to  occur  in 
mountainous  regions,  and  particularly  in  regions  in  which  the  water  contains 
a  great  deal  of  salts  of  lime  and  magnesia.  But  this  is  evidently  not  the 
only  explanation,  for  the  children  of  persons  who  have  emigrated  from  such 
districts  have  often  been  affected  with  the  disease  although  subject  to  en- 
tirely different  atmospheric  and  tellurian  conditions.  The  frequent  intermar- 
riages between  people  in  isolated  mountainous  districts  also  has  its  bearing 
upon  the  hereditary  transmission  of  these  diseases.* 

Diagnosis. — Myxedematous  idiocy  cannot  very  well  be  mistaken  for 
any  other  condition.  It  is  only  in  the  earlier  years  of  life,  when  the  stunted 
growth  and  the  peculiar  expression  of  the  child  are  not  yet  fully  marked,  that 
the  true  cause  of  the  idiocy  may  not  be  suspected.  But  the  arrest  in  the 
general  growth  and  the  peculiar  appearance  of  the  skin  will  give  the  clew  to 
the  true  state  of  affairs.f 

Prognosis. — The  prognosis  of  this  condition  was  extremely  grave  until 
very  recently,  and  therapeutic  experiments  are  now  proceeding  which  will 
prove  whether  this  one  class  of  idiots  may  possibly  be  benefited  by  treatment. 
Until  a  decade  or  more  ago  there  was  as  little  hope  of  improving  myxoedematous 
patients  as  of  helping  other  congenital  idiots,  and  a  removal  from  the  moun- 
tainous region  in  which  cretinism  was  endemic,  or  an  entire  change  of  sur- 
roundings, was  supposed  to  be  the  only  possible  way  of  procuring  some  relief, 
though  actual  cure  of  any  such  condition  had  not,  to  my  knowledge,  been 
recorded  by  anyone. 

As  will  be  seen  in  the  discussion  of  the  treatment  of  this  condition  there 
is  some  hope  of  improving  these  children  by  the  use  of  recent  methods. 

Treatment. — All  the  older  suggestions  with  regard  to  treatment  can 
be  discarded.  Feeding  the  patient  with  the  thyroid  gland  promises  some  re- 
lief. The  author  has  been  personally  interested  in  the  treatment  of  several 
patients  with  myxoedematous  idiocy.  In  one  of  them  a  very  decided  change 
was  effected,  the  mental  and  physical  symptoms  being  greatly  benefited  by 
the  use  of  thyroid  extract.  In  two  other  cases  the  treatment  was  entirely 
unsuccessful,  and  in  a  fourth  patient,  a  cretin,  aged  thirty-four  years,  a  con- 
siderable change  in  the  physical  condition  was  effected,  but  the  patient 
became  so  emaciated  that  the  drug  had  to  be  discontinued.^  The  pulverized 
gland  is  now  for  sale  in  the  market  and  can  be  obtained  easily  in  any  quan- 
tity. With  children  the  treatment  should  be  begun  with  doses  of  one  grain 
of  the  thyroid  gland,  to  be  given  three  times  a  day,  and  increased  from  one 

*  For  the  pathology  of  this  condition,  see  page  199. 

t  These  conditions  bear  a  superficial  resemblance  to  infantilism — a  condition  char- 
acterized by  stunted  growth,  a  persistence  of  child-like  physique,  non-development  of 
the  genital  organs,  and  of  those  physical  and  mental  traits  which  go  with  normal  sexual 
growth  (Bourneville,  Brissaud,  Meige,  Lorain,  and  other  French  and  Italian  writers). 
There  is  some  resemblance  also  to  achondroplasia— a  stunted  growth  of  the  extremities 
with  normal  growth  of  the  trunk  (Marie).  Skull  relatively  large.  The  epiphyses  are 
deformed  and  thickened.     Intelligence,  as  a  rule,  remains  normal. 

X  All  these  cases  were  treated  more  than  ten  years  ago.  With  the  improved 
method  of  administering  the  "gland,"  better  results  have  been  obtained. 


IDIOCY  AND  IMBECILITY.  557 

up  to  five  grains.  While  the  drug  is  being  given  the  child  should  be  under 
proper  medical  observation,  and  the  effect  of  the  administration  of  the  gland 
upon  the  heart  and  other  organs  should  be  carefully  noted.  This  treatment 
should  be  begun  in  the  earliest  stages  of  myxcedematous  idiocy,  with  the  view 
to  prevent,  if  possible,  the  full  development  of  the  disease. 


APPENDIX. 


APPENDIX. 


56, 


Localization    of    the    Functions    of    the    Segments    of    the 

Spinal  Cord. 

For  reference,  the  following  tables,  as  revised  by  Starr,  are  appended. 


Segment. 

Muscles. 

Reflex. 

Sensation. 

II.     and 

Sterno-mastoid. 

Hypochondrium  (?). 

Back    of  head  to 

III.  C. 

Trapezius. 

Sudden    inspiration    pro- 

vertex. 

Scaleni  and  neck. 

duced  by  sudden  press- 

Neck. 

Diaphragm. 

ure   beneath  the  lower 
border  of  ribs. 

IV.  C. 

Diaphragm. 

Pupil.  4th  to  7th  cervical. 

Neck. 

Deltoid. 

Dilatation    of    the  pupil 

Upper  shoulder. 

Biceps. 

produced    by  irritation 

Outer  arm. 

Coraco-brachialis. 

of  neck. 

- 

Supinator  longus. 

Rhomboid. 

Supra-  and  infra-spinatus. 

V.  c. 

Deltoid. 

Scapular. 

Back  of  shoulder 

Biceps. 

5th  crevical  to  1st  dorsal. 

and  arm. 

Coraco-brachialis. 

Irritation  of  skin  over  the 

Outer  side  of  arm 

Brachialis  anticus. 

scapula  produces  con- 

and forearm, 

Supinator  longus. 

traction  of  the  scapular 

front  and  back. 

Supinator  brevis. 

muscles. 

Rhomboid. 

Supinator  longus. 

Teres  minor. 

Tapping   its    tendon   i  n 

Pectoralis  (clavicular 

wrist   produces  flexion 

part). 

of  forearm. 

Serratus  magnus. 

VI.  c. 

Brachialus  anticus. 

Triceps. 

Outer  side  of  fore- 

Serratus magnus. 

5th  to  6th  cervical. 

arm,  front   and 

Triceps. 

Tapping    elbow    tendon- 

back. 

Extensors    of    wrist  and 

produces    extension  of 

Outer    half     of 

fingers. 

forearm. 

hand. 

Pronators. 

Posterior  wrist. 
6th  to  .8th  cervical. 
Tapping  tendons  causes 
extension  of  hand. 

VII.  c. 

Triceps  (long  head). 

Anterior  wrist. 

Inner    side    and 

Extensors    of    wrist  and 

7th  to  8th  cervical. 

back  of  arm  and 

fingers. 

Tapping  anterior  tendons 

forearm. 

Pronators  of  wrist. 

causes  flexion  of  wrist. 

Outer  half  of  the 

Flexors  of  wrist. 

Palmar.     7th  cervical  to 

hand. 

Subscapular. 

1st  dorsal. 

Pectoralis  (costal  part). 

Stroking    palm  c  a  u  a  es 

Latissimus  dorsi. 

closure  of  fingers. 

Teres  major. 

VIII.  c. 

Flexors    of       wrist    and 

Forearm    and 

fingers. 

hand,  inner  half. 

Intrinsic  muscles  of  hand. 

562 


APPENDIX. 


Localization  of  the  Functions  of  the  Segments  of  the  Spinal  Cord.- 

Continued. 


Segment. 

Muscles. 

Reflex. 

Sensation. 

I.  D. 

Extensors  of  thumb. 
Intrinsic  hand  muscles. 
Thenar   and  hypothenar 
eminences. 

Forearm,  inner 

half. 
Ulnar  distribution 

to  hand. 

II.      to 
XII.  D. 

Muscles    of     back    and 

abdomen. 
Erectores  spinae. 

Epigastric.     4th    to    7th 

dorsal. 
Tickling  rnammaryregion 

causes  retraction  of  the 

epigastrium. 
Abdominal.     7th   to  nth 

dorsal. 
Stroking  side  of  abdomen 

causes    retraction    0  f 

belly. 

Skin  of  chest  and 
abdomen,       in 
horizontal 
bands    running 
around    the 
body. 

I.  L. 

Ilio-psoas. 
Sartorius. 
Muscles  of  abdomen. 

Cremasteric.     1st   to   3d 

lumbar. 
Stroking  inner  thigh 

causes    retraction     0  f 

scrotum. 

Skin  over  groin 
and  front  of 
scrotum. 

Over  back  above 
buttocks. 

II.  L. 

Ilio-psoas.     Sartorius. 
Quadriceps  femoris. 
Flexors  of  knee. 

Patella  tendon. 
Striking     tendon    causes 
extension  of  leg. 

Front  of  thigh. 

III>^/ 

•Quadriceps  femoris. 
Flexors  of  knee. 
Inner  rotators  of  thigh. 
Abductors  of  thigh. 

0 

Front   and   outer 
side  of  thigh. 

IV.  L.    i. 

Abductors  of  thigh. 
Adductors  of  thigh. 
Tibialis  anticus. 

Gluteal. 

4th  to  5th  lumbar. 

Stroking  buttock   causes 
dimpling  in  fold  of  but- 
tock. 

Outer  side  of  leg. 

Inner  side  of  thigh 

and  leg  to  ankle. 

Inner  side  of  foot. 

V.  L. 

Outward  rotators  of  thigh. 
Flexors  of  ankle. 
Extensors  of  toes. 

Back  of  thigh, back 
and  outer  part  of 
leg  and  of  foot. 

I.  to  II. 

S. 

Flexors  of  ankle. 

Long  flexor  of  toes. 

Peronei. 

Intrinsic  muscles  of  foot. 

Plantar. 

Tickling  sole   offoot 
causes  flexion  of  toes 
and  retraction  of  leg. 

Back  of  thigh  and 
outer  side  0  f 
foot. 

Buttocks. 

III.  to 
V.  S. 

Perineal  muscles. 

Foot  reflex.     Achilles 
tendon. 

Over-extension   of  f  0  0  t 
causes    rapid    flexion ; 
ankle-clonus. 

Bladder  and   rectal  cen- 
tres. 

Skin  over  sacrum, 
anus,  perineum, 
genital  s,     and 
lower     part    of 
buttocks. 

INDEX. 


INDEX. 


Abducens  nerve,  in  tumor  of  cerebel- 
lum, 479 
Abscess  of  brain,  488 

chronic  abscess  of,  492 

differential  diagnosis,  493 

prognosis,  494 

treatment,  495 

of  cerebellum,  490 
Acute   ascending    paralysis.     See   Lan- 
dry's paralysis,  283 
Agenesis  corticalis,  399,  513 
Agoraphobia,  524 
Amaurotic  family  idiocy,  462 

symptoms,  463 

etiology,  464 

diagnosis,  465 

pathological  anatomy,  465 

prognosis,  468 

treatment,  469 
Amyelia,  386 

Amyotrophic  lateral  sclerosis  with  bul- 
bar symptoms,  420 
Amyotrophies  and  myopathies,  distinc- 
tion between,  353-354 
Anaemia,  headaches  due  to,  167 
Anaesthesia,  37 

hysterical,  98 
Aneurism,  cerebral,  484 
Angio-neurotic  oedema,  201 
Angio-paralytic,  angio-spastic  forms  of 

migraine,  179 
Ankle  clonus,  40 
Anosmia   (loss    of   smell)    in   tumor  of 

frontal  lobe,  475 
Antitoxin  of  tetanus,  152 
Aphasia,  439 

aphasia  in  migraine,  177 
Aphonia,  hysterical,  95 
Appendix,  561 

Aran-Duchenne  type  of  progressive  mus- 
cular atrophy,  353-355 
Arm,  palsies  of,  210 
Arsenic,    method    of    administering    in 

chorea,   126 
Astasia-abasia,  94 
Atelomyelia,  386 

Athetosis,  442,  445.     See  also  "Double 
athetosis." 


Atrophy.     See      progressive     muscular 

atrophies. 
Auditory  nerve.     See  Hearing. 
Auditory  symptoms  in  epilepsy,  67 
Aura,  in  epilepsy,  69 

Basal  Ganglia,  tumor  of,  477 
Basedow's  disease,  192 

cardinal  symptoms  of,  192 

theories  of,  196 

treatment  of,  197 
Baths,  efficacy  of,  in  chorea,  125 
Bell's  paralysis,  216 
Birth  palsies,  436 

morbid  anatomy  of,  447 
Blepharospasm,  225 
Blood-vessels,  dilatation  of,  in  the  brain 

in  chorea,  120 
Bouche  de  tapir  in  progressive  muscular 

atrophy,  375 
Brain  abscess,  488 

symptomatology  of,  491 

defective  development  of,  501 

development  of,  1 

diseases  of,  391 

hypertrophy  of,  514 

of  tumors  in  the,  470 
Bromides,  mode  of  administering  in  epi- 
lepsy, 81 
Brown-Sequard's  paralysis,  with  tumors 
of  spinal  cord,  323 

in  syphilis  of  the  spinal  cord,  294 
Bulbar  palsies,  418 

Caput  obstiptjm,  15 

Catalepsy,  94 

Cephalic  tetanus,  147    . 

Cerebellar  type  of  hereditary  ataxy,  344 

Cerebellum,  tumor  of,  479 

Cerebral  development,  arrest  of,  462, 
501,  446 

Cerebral  diplegia,  family  forms  of,  469 

Cerebral  neurasthenia,  524 

Cerebral  palsies,  430 

Cerebral  tumors,  470 

Cerebral  tyre  of  hereditary  spastic  par- 
alysis, 345-446  _ 

Cerebro-spinal  meningitis,  404 


565 


566 


INDEX. 


Cervical  segments  in  plexus  lesions.    See 

appendix,  561 
Charcot-Marie  type  of  progressive  mus- 
cular atrophy,  359 
Chorea,  108 

complications  of,  116 

duration  of,  117 

electrical  reactions  in,  115 

germ  theory  of,  112 

hereditary  form  of,  129 

hygienic  care  in,  125 

mental  disturbance  in,  115 

morbid  anatomy    and    pathology    of, 
119 

relapses  in,  123 

treatment  of,  124 
Chorea  electrica,  136 
Chorea  insaniens,  115 

of  Sydenham,  108 
Choreiform  diseases,  129 
Choreiform   movements,   after  cerebral 

palsies,  442 
Circular  insanity,  532 
Clonus,  ankle,  40 
Color-sense,  in  hysteria,  100 
Complex  tics,  134 
Compression  of  spinal  cord,  315 
Congenital  diplegias,  350,  439 
Congenital  paramyotonia,  141 
Conjugated  paralysis,  12 
Contracture  in  cerebral  palsies,  440 
Contracture  in  myelitis,  275 
Contracture    rheumatismal    des    nour- 

rices,  156 
Convulsions,  51 

causes  of,  52 

diagnosis  of,  62 

frequency  of,  56 

in  epilepsy,  68 

in  hysteria,  89 

in  lead-poisoning,  247 

partial,  significance  of,  57 

prognosis,  63 

theories  of,  55 

treatment  of,  64 
Convulsive  tremor,  142 
Coprolalia,  225 
Cornu  ammonis,  disease  of,  as  a  cause 

of  epilepsy,  77 
Corpora  quadrigemina,  tumor  of,  477 
Cortical  tumors,  474 
Cranial  measurements,  5 
Cranial  nerve  nuclei,  defective  develop- 
ment of,  515 
Craniotabes,  60 

Craniotomy,  in    infantile  cerebral   pal- 
sies, 460 

inidiocy,  554 
Cretinism,  sporadic,  554 
Crus  cerebri,  tumor  of,  477 
Cyclops,  501 


Cysts    of    brain    in    infantile    cerebral 
palsy,  450 

Defective  development  of  brain,  501 
Deformities  in  infantile  spinal  paralysis, 

256 
Degeneration,  in  the  brain, 

in  idiocy,  548 

in  insanity,  540 
Degeneration,  electrical  reaction,  44 
Dementia,  533 
Dementia  prascox,  533 

in  hereditary  chorea,  130 
Diet,  in  chorea,  125 
Dietary,  in  epilepsy,  83 
Diphtheretic  paralysis,  240 

course  and  duration  of,  242 

pathological  anatomy  of,  243 
Diplegia,^;  y.^ 
Diplomyelia,  307 
Diplopia,  12 
Disseminated  sclerosis,  304 

atypical  forms  of,  310 

differentiation  from  paralysis  agitans, 

.3" 

etiology  of,  307 

pathology  of,  308 

prognosis  of,  313 

symptoms  of,  304 

treatment  of,  313 
Dolichocephalus,  6 
Double  athetosis,  446 
Duchenne's  type,  353-355 
Dystrophies,  progressive  muscular,  368 

Ear  Disease,  as  a  cause  of  headache, 
171 ;  of  abscess,  489;  of  sinus  throm- 
bosis, 499 
Echolalia,  137 
Eclampsia  infantum,  51 
Electrical  conditions,  table  of,  44-45 

examination,  42 

examination    in    progressive    neural 
muscular  atrophy,  362 

reactions  in  chorea,  115 

reactions     in     progressive     muscular 
atrophies,  355_377- 
Electricity,  service  of,  45 

use  of,  in  chorea,  127 
Embolism  in  chorea,  121 
Encephalitis,  acute,  391,  416 

hermorrhagic,  acute,  416 
Encephalocele,  501 
Enuresis  nocturna,  191 
Epidemic  cerebro-spinal  meningitis,  404 

symptoms  of,  407 
Epilepsia  nutans,  135 
Epilepsy,  67 

aurae  in,  69 

causes  of,  69—71 

diagnosis  of,  74 


INDEX. 


567 


Epilepsy,  Jacksonian,  69.     See  Partial 
Convulsions. 

pathological  anatomy  of,  77 

pathology  of,  79 

prognosis  of,  76 

procursive,  71 

psychic  symptoms  of,  69-70 

surgical  treatment  of,  83 

symptoms  of,  67 

temperature  in,  71 

treatment  of,  80;    Flechsig's  method, 
82 

varieties  of,  66 
Epileptic  aura,  treatment  of,  83 
Epileptic  insanity,  537 
Epileptic  equivalents,  68 
Epileptiform  convulsions,  diagnosis  from 

chorea,  119 
Erb's  palsy,  210 

Erb's  type  of  progressive  muscular  atro- 
phy, 374 
Essential  paralysis  of  children,  249 
Examination,  method  of,  1 

scheme  of,  3 
Exophthalmic  goitre,  192 
Eye-strain,  cause  of  headache,  174;    re- 
lation to  migraine,  181 
Eyes,  conjugate  deviation  of,  9 

muscles  of,  10 

Face,  asymmetry  of,  12 

motor  points  of,  17 
Facial  hemiatrophy,  204 
Facial  palsy,  216 

a  form  of  obstetrical  palsy,  215 

electrical  changes  in,  218-219 

treatment  of,  223 
Facies  myopathique  in  progressive  mus- 
cular atrophy,  374 
Facio-scapulo-humeral  type  of  progres- 
sive muscular  atrophy,  374 
Flechsig's  treatment  of  epilepsy,  82 
Friedreich's  disease,  .334 

diagnosis  of,  from  hereditary  chorea, 

I31 
differential  diagnosis  of,  339 
pathological  anatomy  of,  340 
reflexes  in,  337 
symptoms  of,  335 

Gait,  examination  of,  35 

Gangrene,  symmetrical,  203 

Gastric  disturbances,  headache  due  to, 

171 
Genital  irritation,  headaches  due  to,  171 
Glioma  of  brain,  570 
Gliosis  of  spinal  cord,  329 
Glosso-labio-pharyngeal   paralysis,   420 
Glycosuria,  203 
Graefe's  symptom,  194 
Grand  mal,  68 


Graves's  disease,  192 
Gyrospasms,  135 
causes  of,  135 

Habit  chorea,  134 
Hemoglobinuria  intermittent,  203 
Headaches,  166 

a  symptom  of  brain  tumor,  558 

classification  of,  166 

in  organic  disease  of  the  brain,  172 
Head  pains,  location  of,  167 
Hearing,  examination  of,  12 
Heart,  affections  of,  in  chorea,  116 
Hebephrenia,  533 
Hemianesthesia,  40 

hysterical,  98 
Hemianopsia,  9 

in  cerebral  palsies,  439 
Hemiatrophy  of  face,  204 
Hemichorea,  113-117 
Hemicrania,  175 

Hemiplegia,  430,  433,  441,  449,  542 
Hemorrhage,  cerebral,  449 
Hereditary  ataxy,  334 
Hereditary  chorea,  129 

differential  diagnosis  from  post  hemi- 
plegic  chorea,  131 

pathological  anatomy  of,  132 

symptoms  of,  130 
Hereditary  chorea,    without    dementia, 

133 
Hereditary  diseases  of  spinal  cord,  331 
Hereditary  spastic  paralysis,  345 
the  cerebral  type  of,  345,  446 
spastic  paralysis  of  spinal  origin,  345- 

35° 
Heredity,  influence  of,  in  insanity,  520 
Heredo-ataxie  cerebelleuse,  344 
Heterotopia,  386 
Huntington's  chorea,  129;  prognosis  in, 

133 

treatment  of,  133 
Hydrencephalocele,  501 
Hydrocephalus,  420 

a  cause  of  epilepsy,  77 

acquired  internal,  420—426 

chronic,  423 

congenital,  424 

indications  of,  6 

operative  procedures  for,  428 
Hydrorrachis  externa,  387 
Hygiene,  importance  of,  in  chorea,  125 
Hypsesthesia,  37 

Hyperaemia,  headaches  due  to,  170 
Hyperesthesia,  40 
Hypertrophic  and  progessive  interstitial 

neuritis  of  childhood,  385 
Hypochrondriasis,  526 
Hypoglossal  nerve,  spasm  of,  226 
Hysteria,  86 

diagnosis  of,  102 


568 


INDEX. 


Hysteria,  duration  of,  103 

irregular  manifestations  of,  92 

manifestations  of,  87 

motor  manifestations  of,  89 

muscular  paralysis  in,  94 

sensory  symptoms,  97 

pathology  of,  103 
Hysteria,  psychic  or  mental,  87 

treatment  of,  104 

visceral  symptoms  of,  100 
Hysterical    anaesthesia,  transference  of, 

99 
anorexia,  100 
chorea,  differentiation  from  hereditary 

chorea,  132 
insanity,  537 

paralysis,    differentiation   from   acute 
myelitis,  284 
Hystero-epilepsy,  diagnosis  of,  92 

relationship  to  true  epilepsy,  88 
Hysterogenic  zones,  99 

Idiocy,  541 

acquired,  543 

classification  of,  542 

developmental,  543 

hereditary  congenital,  542 

signs  of  degeneration  in,  548 

with  epilepsy,  444 

amaurotic  family,  462 
Imbecility,  541 

Imperative  conceptions,  139,  522 
Imperative  movements,  135 
Infantile  cerebral  palsies,  430 

classification  of,  447 

differential  diagnosis,  455 

distribution  of,  433 

due  to  embolism  and  thrombosis,  449- 

45  2 
due  to  hemorrhage,  449 
etiology  of,  435 
form  of  palsy,  438 
morbid  anatomy,  446 
prognosis  of,  456 
treatment,  457 
with  epilepsy,  444 
with  idiocy,  444 
Infantile  oculo-facial  palsy,  516 
Infantile  spinal  paralysis,  249 

morbid    anatomy  and    pathology   of, 

258 
prognosis  in,  267 
symptoms  of,  249 
theory  of,  263 
treatment  of,  268 
Infectious  disease,  the  cause  of  menin- 
gitis, 414 
headache  due  to,  173 
Inflammation  of  nerves,  209,  238 
Influenza,  the  cause  of  meningitis,  415 
of  encephalitis,  416 


Insanity,  519 

etiology  of,  520 

forms  of,  522 

frequency  of,  520 

treatment  of,  540 
Insomnia,  186;   in  polyneuritis,  239 
Insufficiencies,  ocular,  relation  to  head- 
ache, migraine,  174,  181 
Intra-cranial  sinuses,  thrombosis  of,  497 

Jacksonian  (partial)  epilepsy,  69 
Jaw-jerk,  40 
Jumpers,  138 

Juvenile  form  of  progressive  muscular 
atrophy,  374 

Katatonia,  533 
Knee-jerk,  40 

Landotjzy-Dejerine  type  of  progres- 
sive muscular  atrophy,  374 

Landry's  paralysis,  differentiation  from 
multiple  neuritis,  237 
differentiation  from  acute  myelitis,  283 

Lannelongue's  operation,  509 

Laryngeal  chorea,  114 

Laryngismus  stridulus,  59 

Lead  encephalopathy,  247 

Lead  paralysis,  246 

Leg,   palsies   of,   225.     See  also  Para- 
plegia. 

Leg  type  of  progressive  muscular  atro- 
phy, 359 

Lenticular  nucleus,  affection  of,  in  cho- 
rea, 122 

Leptomeningitis.     See  meningitis. 

Leptomeningitis,  specific,  293 

Lid  reflex,  light  reflex,  10 

Little's  disease,  diagnosis  of,  from  hered- 
itary spastic  paralysis,  350 

Localization  in  spinal  cord.     (See  Ap- 
pendix), 561 
of  tumors,  474 

Lumbar  puncture,  46 

Macewen's  symptom,  474 
Macrocephalus,  514 
Main  en  griffe,  27 
Maladie  des  tics  convulsifs,  137 
Malaria,  headaches  due  to,  173 
Malformations   and   defective   develop- 
ment of  the  cord,  386;   of  the  brain, 

589 
Mania,  547 

Masturbation  and  insanity,  539 
Megrim,  175.     See  Migraine. 
Melancholia,  529 

attonita,  531 
Membranes,    diseases   of.     See  Menin- 
gitis and  other  diseases  of  brain. 


INDEX. 


569 


Membranes  of  the  spinal  cord,  314 

tumors  of,  321 
Meningeal  hemorrhage  at  birth,  448 
Meninges.     See  Membranes. 
Meningitis,  391 

acute,  391 

chronic  basilar,  415 

epidemic,  404 

forms  of,  391 

infectious  diseases,  391 

and  influenza,  415 

and  otitis  media,  412 

serosa,  421 

traumatism,  411 

treatment  of,  395,  403,  410 

tubercular,  397 
Meningocele,  388,  501 
Meningo-myelitis,  specific,  293 
Meningo-myelocele,  388 
Mental  disturbance  in  chorea,  115 
Mental  excitement,  control  of,  in  chorea, 

127 
Microcephalus,  507 
Micromegaly,  387 
Migraine,  175 

coular  insufficiencies  in,  181 

diagnosis  of,  182 

etiology  of,  178 

pathology  of,  179 

periodicity  of,  179 

prognosis  of,  182 

relation  of,  to  epilepsy,  180 

treatment  of,  183 
Moral  insanity,  536 
Motor  area,  tumor  of,  475 
Motor  points,  17,  23,  25,  29,  32,  33 
Multiple  neuritis,  227 

forms  of,  233 

pathological  anatomy  of,  235 

symptoms  of,  227 

treatment  of,  238 
Multiple  sclerosis,  diagnosis  from  Fried- 
reich's disease,  340.     See  also  dis- 
seminated sclerosis. 
Muscles  of  arm,  forearm,  and  hand,  22 

of  back  and  trunk,  33 

of  the  head  and  neck,  15 

of  the  eyes  and  face,  10,  11 

of  pelvic  girdle  and  lower  extremities, 
28,  30 

of  shoulders  and  upper  extremity,  16 

of  the  tongue,  palate,  and  pharynx,  13 

total  absence  and  early  atrophy  of,  384 
Muscular    hypertrophy,     physiological, 

377 
irritability  in  Thomsen's  disease,  140 
movements  in  hereditary  chorea,  130 

Muscular  pseudo-hypertrophy,  371 

Muscular  sense,  37 

Muscular  strength,  tests  of,  34 

Mutism,  hysterical,  95 


Mydriasis,  11 
Myelitis,  acute,  272 

differential  diagnosis,  282 

pathology  and  morbid  anatomy  of,  278 

specific,  293 

symptoms  of  acute  form,  272 

treatment  of,  285 
Myopathies,  268 

diagnosis,  377 

pathology,  379 

treatment,  383 
Myosis,  causes  of,  12 
Myotonia  congenita,  139 
Myotonic  reaction,  140 
Myoclonus  epilepsy,  142 
Mysophobia,  523 
Myxcedema,  199 

treatment  of,  201 
Myxcedematous  idiocy,  201,  544 

Nerves,  cranial,  degeneration  and  in- 
flammation of,  209 
facial,  disease  of,  216 
motor,  of  the  eye,  10 
of  arm,  forearm,  and  hand,  22 
of  back  and  trunk,  n 
of  head  and  neck,  15 
of  pelvic  girdle  and  lower  extremities, 

36 
of  shoulders  and  lower  extremities,  16 
of  tongue,  palate,  and  pharynx,  14 

Nerve-trunks,  inflammation  of,  209 

Nervous  system,  organic  diseases  of,  206 
general  functional  diseases,  48 

Neurasthenia,  cerebral  type  of,  524 
headache  due  to,  169 

Neuritis,  227   240      See  also  385. 

Neuroglia,  hyperplasia  of,  in  epilepsy,  78 

Nictitation,  225 

Nonne-Marie  type  of  cerebellar  ataxy, 

344 

Nystagmus,  9 

Obstetrical  palsies,  210 

diagnosis  of,  212 

pathology  of,  211 

treatment  of,  214 
Occipital  lobe,  defective  development  of, 

511 
tumor  of,  476 
(Edema,,  anigo-neurotic,  201 
Ophthalmoplegia,  externa  and  interna, 
12. 
partial  and  complete,  418 
Opisthotonus,  143,  158 
Optic  thalamus,  affection  of,  in  chorea, 

122 
Otitic  disease,  relation  of,  to  brain  ab- 
scess, 489.     See  Ear  disease. 
I  Oxycephalus,  6 


570 


INDEX. 


Pain,  36 

Palsy.     See  Paralysis. 
Paradoxical  contraction,  40 
Paraesthesiae,  36 

Paralysis,   distribution   of,   in  infantile 
cerebral  palsies,  433 

Erb's,  210 

facial,  216 

infantile  spinal,  249 

in  peripheral  nerve  disease,  209 

in  spinal  palsies,  249 

of  ocular  muscles  in  disseminated  scle- 
rosis, 307 
Paramyoclonus  multiplex,  141 
Paranoia,  534 
Paraplegia,  spastic  cerebral,  430 

spastic  spinal,  347 
Paretic  dementia,  538 
Parietal  lobe,  tumor  of,  476 
Patellar  reflex,  40 
Pavor  nocturnus,  188 
Periodic  insanity,  532 
Peripheral  nerves,  diseases  of,  209 

palsies,  209,  225 
Peroneal  paralysis,  225 

form  of  progressive  muscular  atrophy, 

359 
Petit  mal,  68 
Pleuroplegia,  516 

congenital,  517 
Polio-encephalitis  superior,  417 
Polio-encephalomyelitis,  418 
Poliomyelitis  anterior  acuta,  249 
Polyneuritis,  238.     See  Multiple  neuri- 

'  tis. 
Pons,  tumor  of,  478 
Porencephaly,  502 

acquired,  505 

congenital,  503 
Post-hemiplegic  chorea,  117,  442 
Pott's  paralysis,  3,  315 

diagnosis  of,  318 

symptoms  of,  317 
Primary  dystrophies,  types  of,  369 

muscular  dystrophies,  368 

myopathies,  368 
Progressive  amyotrophy,  355 
Progressive  muscular  atrophies,  352 

pathology  of,  356 

hereditary  form,  357 

muscular  atrophy,  abortive  and  hered- 
itary forms  of,  359 
Progressive    neural    muscular    atrophy, 

359 
neurotic  muscular  atrophy,  359 
pathology  of,  366 
Pseudo-hypertrophic   muscular   paraly- 
sis, 371 
Pseudo-bulbar  palsy,  420 
Ptosis,  n,  241 
Pupil  inequality,  12 


Raynaud's  disease,  203 
Reaction  of  degeneration,  44 
Reflex  of  cornea,  test  for,  10 
Reflexes,  examination  of,  40 

variations  of,  41 
Rest,  efficacy  of,  in  chorea,  124 
Rheumatic  nodules  in  chorea,  116 
Risus  sardonicus,  145 

Sarcoma  of  brain,  483 
Scaphocephalus,  6 
Sclerosis,  amyotrophic  lateral,  420 
Sensation,  disturbances  of,  in  migraine, 
176 

tests  for,  37 

varieties  of,  37 
Sensory  disturbances,  determination  of, 

36,  37 
Sensory  nerves,  distribution  of,  38,  39 
Skull,  abnormalities  of,  6 

measurements  of,  4 
Sleep,  disorders  of,  186 

normal  amount  of,  187 
Somnambulism,  191 
Spasms,  135  et  seq. 
Spastic  hemiplegia,  430 

diplegia,  396,  430 

paraplegia,  430 

spinal  paralysis,  347 
Speech,  difficulties  of,  in  maladie  des  tics 

convulsifs,  138 
Speech,   disturbance   of,   in   hereditary 
chorea,  131 

disturbances  of,  in  chorea,  114 
Spina  bifida,  387 
Spinal  cord,  gliosis  of,  329 

hereditary  or  family  diseases  of,  331 

injuries  of,  288 

localization  in.     See  Appendix. 

malformations  and  conditions  due  to 
defective  development  of,  386 

syphilis  of,  293 

tumors  of,  321 
Spotted  fever,  404 
Stellwag's    symptom    in    exophthalmic 

goitre,  194 
Subacute  anterior  poliomyelitis,  270 
Subjective  sensations,  36 
Sydenham's  disease,  108 
Syphilis  of  spinal  cord,  morbid  anatomy 
of,  299 

symptoms  of,  293 

treatment  of,  302 
Syringomyelia,  329 
Syringo-myelocele,  388 

Tabes  dorsalis  spasmodique,  350 
Tachycardia,  199 

Teeth,  significance  of  condition  of,  13 
Temperature-sense,  37 
Temporo-sphenoidal  lobe,  tumor  of,  476 


INDEX. 


57' 


Tendon  reflexes,  40 
Tetanilla,  156.     See  Tetany. 
Tetanoid  chorea,  164 
Tetanus,  143 

bacillus  of,  150 

differential  diagnosis  of,  152 

etiology  of,  141 

pathology  of,  149 

prognosis  of,  153 

symptoms  of,  145 

treatment  of,  153 

varieties  of,  147 
Tetanus  neonatorum,  143,  147 
Tetany,  156 

etiology  of,  159 

latent  period  of,  160 

pathology  of,  161 

symptoms  of,  157 

treatment  of,  163 
Thomsen's  disease,  139 
Thrombosis,    of    intra-cranial    sinuses, 

497 

of  lateral  sinus,  differential  diagnosis 
from  brain  abscess,  499 
Thyroid  enlargement  at  puberty,  199 
Thyroid  gland,  extirpation  of,  in  relation 
to  tetany,  162 

feeding  in  myxcedema,  201,  544 
Tic  convulsif,  137,  225 
Tics,  134 
Topoalgia,  36 
Torticollis,  spasmodic,  225 
Toxic  headaches,  174 

injuries  of  spinal  cord,  288 
Tremor,  fibrillary,  354 

of  multiple  sclerosis,  304 
Trigeminal  neuralgia,  rarity  of,  182 
Trismus,  143 

Trophic  disturbances  in  peripheral  neu- 
ritis, 209 
Trophic  symptoms,  occurrence  of,  45 
Tropho-neuroses,  192 
Trousseau's  symptom,  160 
Tubercular  meningitis,  397 

morbid  anatomy,  400 


Tubercular  meningitis,  onset,  397 

symptoms,  397 
Tumor  of  cortex,  474 

of  basal  ganglia,  477 

of  cerebellum,  479 

of  corpora  quadrigemina,  477 

of  eras  cerebri,  477 

frontal  lobe,  475 

of  medulla  and  pons,  478 

of  motor  area,  475 

of  occipital  lobe,  476 

of  parietal  lobe,  476 

of  temporo-sphenoidal  lobe,  476 

of  third  frontal  convolution,  475 
Tumors  of  the  brain  and  meninges,  470 

differential  diagnosis,  480 

pathology  of,  481 

treatment  of,  484 
Tumors  of  spinal  cord  and  meninges, 
320 

diagnosis  of,  325 

prognosis,  327 

symptoms  ®f,  320 

treatment  of,  327 


Ue.emia,  headaches  due  to,  173 
Urine,  changes  in,  in  chorea,  116 


Vasomotor  neuroses,  192 

Ventricle,  fourth,  stimulation  of  floor  of, 

Vertigo  in  migraine,  177 
Vision,    disturbances    of,    in    hysteria, 
100 
test  for,  in  infants,  8 
Visual  field,  9 

Weight-sense,  37 

Weir-Mitchell   plan   of   treatment,    105 

Wrist-drop,  21,  241 

Wry-neck,  225 

congenital,  225 

symptomatic,  225 


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